carnitine and s-adenosylmethionine

carnitine has been researched along with s-adenosylmethionine in 1098 studies

Research

Studies (1,098)

TimeframeStudies, this research(%)All Research%
pre-19906 (0.55)18.7374
1990's35 (3.19)18.2507
2000's248 (22.59)29.6817
2010's593 (54.01)24.3611
2020's216 (19.67)2.80

Authors

AuthorsStudies
Jakobs, C; Roe, CR; ten Brink, HJ; Verhoeven, NM; Wanders, RJ1
Evengard, B; Kanakura, Y; Kawata, S; Kitani, T; Kuratsune, H; Långström, B; Lindh, G; Machii, T; Matsumura, K; Takahashi, M; Takaishi, J; Watanabe, Y; Yamaguti, K1
Becker, LE; Bennett, MJ; Haslam, RH; Rhead, WJ; Tein, I; Vockley, J1
Harashima, H; Hayashi, M; Horiuchi, M; Kamido, H; Kudo, T; Kuwajima, M; Lu, K; Ono, A; Ozaki, K; Saheki, T; Sei, M; Shima, K1
Brühlmann, P; Hoppeler, H; Krähenbühl, S; Stucki, G; Willer, B1
de Hoffmann, E; Libert, R; Nassogne, MC; Schanck, A; Stroobant, V; Thillaye, M; Van Hoof, F; Vincent, MF1
Bonham, JR; Downing, M; Edwards, RG; Manning, NJ; Olpin, SE; Pollitt, RJ; Pourfarzam, M; Sharrard, MJ; Tanner, MS1
Matsumoto, Y1
Bayés, R; Campoy, C; López, C; Molina-Font, JA; Peinado, JM; Rivero, M1
Allers, P; Costa, CG; Duran, M; Ijlst, L; Jakobs, C; Ruiter, J; Struys, EA; Tavares de Almeida, I; Ventura, FV; Wanders, RJ1
Alhomida, AS1
al Aqeel, AI; Rashed, MS; Wanders, RJ1
Bootsma, AH; Overmars, H; van Gennip, AH; van Lint, AE; Vreken, P; Wanders, RJ2
Hillman, SL; Matern, D; Mayatepek, E; Millington, DS; Strauss, AW; Trefz, FK1
Gibson, B; Hahn, SH; Hong, CH; Jung, JW; Lee, EH; Rinaldo, P; Sims, H; Strauss, AW; Yoon, HR1
Haseler, LJ; Karpinski, NC; Terkeltaub, RA; Videen, JS1
Carregal, M; Cerruti, S; Dalamon, V; Savino, EA; Testoni, G; Varela, A1
Carter, AL; Ganapathy, ME; Ganapathy, V; Hopfer, U; Huang, W; Leibach, FH; Shaikh, SN1
Adachi, Y; Azuma, A; Hirasaki, S; Ito, K; Kinoshita, N; Kodo, N; Matsuo, A; Nakagawa, M; Nakamura, T; Sugihara, H1
Inoue, F; Kinugasa, A; Kizaki, Z; Kodo, N; Nakajima, H; Okochi, M; Sawada, T; Terada, N1
Bryant, SD; Doncel, GF; Gandour, RD; Hubieki, MP; Robinette, RG; Savle, PS1
Costa, CC; de Almeida, IT; Duran, M; Jakobs, C; Poll-The, BT1
Aleck, K; Ding, JH; Grebe, TA; He, G; Roe, CR; Roe, DS; Teramoto, R; Yang, BZ1
Carotti, A; Cellamare, S; D'Acquarica, I; Gasparrini, F; Misiti, D; Muck, S; Villani, C1
Bonnet, D; Brivet, M; Jouvet, P; Martin, D; Rabier, D; Saudubray, JM; Villain, E1
Jackson, JM1
Chace, DH; Naylor, EW1
Iacobazzi, V; Lasorsa, FM; Palmieri, F; Palmieri, L; Runswick, MJ; Walker, JE1
Carpenter, K; Wilcken, B; Wiley, V1
Georgala, C; Georgala, S; Michas, T; Schulpis, KH1
Brivet, M; Roe, CR; Roe, DS; Sweetman, L1
Heinig, K; Henion, J1
Bennett, MJ; Chen, YT; Feezor, MD; Hillman, S; Kahler, SG; Matern, D; Millington, DS; Qumsiyeh, M; Shen, JJ; Van Hove, JL1
Bartlett, K; Eaton, S; Markley, MA; New, KJ; Pourfarzam, M; Quant, PA1
Abdenur, JE; Bernard, CI; Chamoles, NA; Guinle, A; Jorge, L; Lavorgna, S; Levandowskiy, V; Schenone, AB; Specola, N1
Cyr, D; Giguère, R; Giguère, Y; Lemieux, B1
de Hoffmann, E; Libert, R; Nassogne, MC; Schanck, A; Thillaye, M; Van Hoof, F; Vincent, MF1
al-Essa, MA; Bakheet, SM; Ozand, PT; Patay, ZJ; Rashed, MS1
Andresen, BS; Korall, H; Schwahn, B; Spiekerkötter, U; Trefz, FK; Wendel, U1
Burlingame, TG; Gibson, KM; Hogema, B; Jakobs, C; Kiss, D; Linck, L; Millington, D; Pohowalla, P; Rinaldo, P; Roe, CR; Roe, DS; Sacks, M; Schutgens, RB; Steiner, RD; Sweetman, L; Vockley, J1
Debreceni, B; Farkas, V; Fischer, GM; Laszlo, A; Nemeti, B; Sandor, A; Schaffer, Z; Somogyi, C1
Bleijenberg, G; Elving, LD; Janssen, AJ; Soetekouw, PM; van der Meer, JW; van der Veen, Y; Vreken, P; Wevers, RA1
Dorland, L; Duran, M; IJlst, L; Muntau, AC; Roscher, AA; Röschinger, W; Wanders, RJ1
Arnecke, R; Balling, R; Dame, T; Hrabé de Angelis, M; Kreischer, J; Olgemöller, B; Rolinski, B; Roscher, AA; Wolf, E1
Kanter, EM; Newatia, A; Yamada, KA1
Deutsch, J; Kalderon, B; Purdon, AD; Rapoport, SI1
Brass, EP1
Baillet, L; Esser, V; McGarry, JD; Mullur, RS1
Han, IK; Heo, K; Lin, X; Odle, J1
Feezor, MD; Hart, P; Kahler, SG; Matern, D; Millington, DS; Ramakrishna, JP; Shen, JJ; Treem, WR; Van Hove, JL1
Arenas, J; Börnstein, B; Campos, Y; de la Calzada, CS; Gómez, MA; Guillén, F; Martín, MA; Rubio, JC1
Krähenbühl, S; Thormann, W; Vernez, L1
Kohlmueller, D; Linderkamp, O; Mayatepek, E; Meyburg, J; Schulze, A1
Blakely, JA; Johnson, MD; Stewart, JM1
Kuratsune, H1
Carpenter, K; Sim, KG; Wilcken, B; Wiley, V2
Adler, S; Amato, A; Brass, EP; Hiatt, WR; Orlando, AM; Sietsema, KE1
Haro, D; Hegardt, FG; Marrero, PF; Nicot, C; Woldegiorgis, G1
Andresen, BS; Banas, R; Bross, P; Chace, DH; Dobrowolski, SF; Engel, P; Frazier, DM; Gregersen, N; Knudsen, I; McCandless, SE; Muenzer, J; Naylor, EW; O'Reilly, L; Udvari, S1
Le, W; Liang, X; Schulz, H; Zhang, D1
Brandt, NJ; Christensen, E; Jensen, UG; Nørgaard-Pedersen, B; Simonsen, H; Skovby, F1
Fiesel, S; Hoffmann, GF; Lindner, M; Mayatepek, E; Olgemöller, K; Penzien, J; Ruiter, JP; Schulze, A; Wanders, RJ; Zschocke, J1
Abdenur, JE; Bernard, C; Chamoles, NA; Fusta, M; Guinle, A; Jorge, L; Lavorgna, S; Levandovskiy, V; Schenone, AB1
Chace, DH; DiPerna, JC; Hofman, LF; Mitchell, BL; Naylor, EW; Sgroi, B1
Bakr, S; Bergmann, SR; Hwang, Y; Ramasamy, R1
Aynsley-Green, A; Berger, R; Clayton, PT; Datta, V; Eaton, S; Edginton, M; Hussain, K; Krywawych, S; Malingre, HE; van den Berg, IE1
Amoroso, A; Boniotto, M; Braida, L; Casetta, B; Crovella, S; de Vonderweid, U; Luchesi, A1
de Almeida, IT; Duran, M; Jakobs, C; Silva, MF; Wanders, RJ1
Carpenter, K; Heath, D; Sim, KG; Wilcken, B; Wiley, V1
Arnecke, R; Dame, T; Fingerhut, R; Kreischer, J; Liebl, B; Muntau, AC; Olgemöller, B; Roscher, AA; Röschinger, W; Superti-Furga, A; Troxler, H1
Roe, CR; Roe, DS; Sharma, S; Vianey-Saban, C; Zabot, MT1
Cox, KB; Hamm, DA; Lindsey, JR; Matern, D; Millington, DS; Pinkert, CA; Rhead, WJ; Rinaldo, P; Vockley, J; Wood, PA1
Kamimori, H; Konishi, M1
Chisholm, CA; Frerman, FE; Lovell, MA; Roe, CR; Roe, DS; Sweetman, L; Vavelidis, F; Wilson, WG1
Baena, RM; Bayés, R; Campoy, C; Goicoechea, A; López, C; Molina-Font, JA; Pedrosa, T; Peinado, JM; Rivero, M1
Hatsutanmaru, K; Masuda, A; Nakayama, T; Tei, C; Yamanaka, T1
de Almeida, IT; Duran, M; Maya, M; Overmars, H; Selhorst, J; Silva, MF; van Gennip, AH; Wanders, RJ1
Carpenter, K; Christodoulou, J; Hammond, J; Sim, KG; Wilcken, B1
Angsten, G; Boberg, M; Cederblad, G; Meurling, S; Stiernström, H1
Bauer, MF; Bieger, I; Gempel, K; Gerbitz, KD; Hofmann, S; Kiechl, S; Kiechl-Kohlendorfer, U; Lochmüller, H; Pongratz, D; Rettinger, A; Sperl, W; Willeit, J1
Kimura, M; Yamaguchi, S1
Green, A; Hardy, D; Preece, MA1
Hansen, EA; Johnson, GS; Katz, ML; Sanders, DA; Sanders, DN1
Kushnir, MM; Pasquali, M; Roberts, WL; Shushan, B1
Can, S; Coker, C; Coker, M; Darcan, S; Gökşen, D; Orbak, Z1
Carpenter, KH; Wiley, V1
Carpenter, K; Christodoulou, J; Giak Sim, K; Hammond, J; Wilcken, B1
Duclos, RI; Hamilton, JA; Ho, JK1
Hoffmann, GF; Kohlmüller, D; Kölker, S; Lindner, M; Mayatepek, E; Okun, JG; Olgemöller, K; Schulze, A; Wanders, RJ1
Champion, MP; Dalton, RN; Turner, C; Wraige, E1
Eggington, M; Kahler, SG; Pitt, JJ1
Al-Hussein, K; Bucknall, MP; Rahbeeni, Z; Rashed, MS; Ruiter, J; Vaz, FM; Wanders, RJ1
Hoffmann, GF; Kohlmueller, D; Linderkamp, O; Mayatepek, E; Meyburg, J; Pöschl, J; Schulze, A1
Cerruti, S; Dalamon, V; Kade, P; Savino, EA; Testoni, G; Varela, A1
Bakr, S; Bergmann, SR; Hwang, YC; Ramasamy, R1
Buehrdel, P; Ceglarek, U; Ethofer, T; Mueller, P; Schindler, I; Schulze, A1
Armstrong, DL; Scaglia, F; Scheuerle, AE; Sweetman, L; Towbin, JA; Wong, LJ1
Brüggemann, L; Kiessling, A; Löster, H; Möder, M1
Hopfgartner, G; Krähenbühl, S; Vernez, L; Wenk, M1
Boenzi, S; Caruso, U; Dionisi-Vici, C; Duran, M; Rizzo, C; Wanders, RJ1
Bailey, KS; Kruckeberg, KE; Matern, D; Nagan, N; Rinaldo, P; Tauscher, AL1
Hoffmann, GF; Kohlmüller, D; Lindner, M; Mayatepek, E; Olgemöller, K; Schulze, A1
Burlingame, T; Connor, WE; Gillingham, MB; Harding, CO; Matern, D; Meeuws, K; Rinaldo, P1
Hata, I; Kimura, M; Sakaguchi, M; Shigematsu, Y; Tokunaga, Y; Watanabe, Y; Yamaguchi, S; Yoshida, I; Yoshino, M1
Andresen, BS; Berry, SA; He, M; Lussky, RC; Madsen, PP; Matern, D; Rinaldo, P; van Calcar, SC; Vockley, J; Whitley, CB; Wolff, JA1
Cordeau, S; Gavino, G; Gavino, VC1
Boney, A; Chaing, S; Frazier, DM; Kishnani, PS; Koeberl, DD; McCandless, SE; McDonald, MT; Millington, DS; Moore, E; Muenzer, J; Smith, WE; Weavil, SD1
Aburatani, H; Fukada, S; Ikeda, K; Matsuura, A; Nimura, Y; Sato, N; Sekoguchi, E; Yasui, A1
Bollaert, PE; de Chillou, C; Feillet, F; Lefebvre, E; Sadoul, N; Steinmann, G; Vianey-Saban, C; Vidailhet, M1
Dixon, MA; Leonard, JV; Lund, AM; Morris, AA; Vreken, P1
Chang, YS; Hwang, SJ; Jin, DK; Lee, JE; Paik, KH; Park, WS; Shim, JW; Strauss, AW; Yoon, HR1
FRITZ, IB; YUE, KT1
BRESSLER, R; FRIEDBERG, SJ1
BRESSLER, R; WITTELS, B1
Arenas, J; Castellano, G; Colina, F; Del Hoyo, P; Martín, A; Martín, MA; Pérez-Carreras, M; Rubio, JC; Solis-Herruzo, JA1
Fukao, T; Hazama, A; Kohno, Y; Kondo, M; Kondo, N; Kubo, T; Matsuo, N; Sakura, N; Shigematsu, Y; Yamaga, H; Yamaguchi, S; Zhang, GX1
Fowler, KJ; Gazeas, S; Ioannou, PA; Kahler, SG; Nefedov, M; Peters, H; Pitt, J; Sarsero, J1
Bali, D; Gregersen, N; Koeberl, DD; Liu, HM; Matern, D; Millington, DS; Stevens, RD; Young, SP1
Chace, DH; Kalas, TA; Naylor, EW1
Bain, MD; Baric, I; Chalmers, RA; Feyh, P; Hoffmann, GF; Jakobs, C; Jeffrey, I; Kölker, S; Okun, JG; Pourfarzam, M; Schor, DS; Wagner, L; Zschocke, J1
Donati, MA; la Marca, G; Malvagia, S; Morrone, A; Pasquini, E; Zammarchi, E1
Arduini, A; Sweeney, JD1
Bocian, M; Boyle, K; Gargus, JJ; Roe, CR; Roe, DS; Vianey-Saban, C1
Davis, AT; Spry, L; Steiber, AL; Weatherspoon, LJ1
An, J; Cline, GW; Fujimoto, Y; Koves, TR; Millington, D; Muoio, DM; Newgard, CB; Shiota, M; Shulman, GI; Stevens, R1
Lehotay, DC; LePage, J; Rockman-Greenberg, C; Thompson, JR1
Duran, M; Exil, V; Mayatepek, E; Spiekerkoetter, U; Strauss, AW; Tokunaga, C; Wanders, RJ; Wendel, U; Wijburg, FA1
Johnson, DW1
Birk, OS; Galron, D; Hershkovitz, E; Kazanovitz, A; Moses, SW1
Krähenbühl, S; Vernez, L; Wenk, M1
Andresen, BS; Brooks, R; Gregersen, N; Gutman, A; Korman, SH; Sinnathamby, T1
Napolitano, N; Pitt, JJ; Wiley, V1
Lai, CC; Lee, CC; Lin, WD; Tsai, FJ; Wang, CH1
Azuero, R; Debata, C; McDonough, K; Penn, D; Quinn, M; Thomson, J1
Sander, S; Sass, JO; Zschocke, J1
Hiatt, WR1
Aubey, F; Bastin, J; Djouadi, F; Schlemmer, D1
Hoppel, CL; Kerner, J; Minkler, PE; North, KN1
Durka, S; Heizmann, CW; Kleinert, P; Kuster, T; Neuheiser, F; Torresani, T; Troxler, H1
Bourdoux, P; Cavedon, CT; de Laet, C; Goyens, P; Herremans, N; Mertens, K; Van Thi, HV1
Liapi-Adamidou, G; Tsagris, V1
Hoppel, CL; Ingalls, ST; Minkler, PE1
Duran, M; Ijlst, L; Mayatepek, E; Overmars, H; Spiekerkoetter, U; Strauss, AW; Tokunaga, C; van Vlies, N; Vaz, FM; Wanders, RJ; Wendel, U; Wijburg, FA1
Bucuvalas, J; Emre, S; Gondolesi, G; Hynan, LS; Magid, M; Morotti, R; Narkewicz, M; Rinaldo, P; Shneider, BL; Squires, R1
Abendschein, DR; Gross, RW; Han, X; Mancuso, DJ; Su, X1
Bodamer, OA; Holub, M; Item, C; Mühl, A; Pollak, A; Stöckler-Ipsiroglu, S1
Chalmers, RA; Harrison, EL; Jones, MG; Jones, S; Lee, PJ; Leonard, JV1
Cline, GW; Coleman, RA; Hammond, LE; Ilkayeva, OR; Muoio, DM; Neschen, S; Romanelli, AJ; Shulman, GI1
Czech, E; Nowicka, J; Olszowy, Z1
Matern, D; Schowalter, DB; Vockley, J1
Andresen, BS; Bischoff, C; Bonham, JR; Chakrapani, A; Clark, S; Downing, M; Gregersen, N; Manning, NJ; Muntoni, F; Olpin, SE; Olsen, RK; Pourfarzam, M; Sharrard, M; Turnbull, DN1
Browning, MF; Larson, C; Marsden, DL; Strauss, A1
Blois, B; Dooley, K; Dyack, S; Riddell, C1
House, R; Lin, X; Odle, J1
Amjad, S; Chalmers, RA; Goodwin, CS; Jones, MG1
Cha, YS; Cho, SW1
Macey, H; Moreno, FA; Schreiber, B1
Eyer, F; Felgenhauer, N; Gempel, K; Gerbitz, KD; Steimer, W; Zilker, T1
Ghoshal, AK; Guo, T; Soldin, SJ; Soukhova, N1
Aubey, F; Bastin, J; Djouadi, F; Ruiter, JP; Schlemmer, D; Strauss, AW; Wanders, RJ1
Gutman, A; Jakobs, C; Korman, SH; Wanders, RJ; Waterham, HR1
Chace, DH; Evans, AM; Faull, RJ; Fornasini, G; Reuter, SE1
Greenberg, CR; Haas, D; Hoffmann, GF; Kohlmüller, D; Kölker, S; Lindner, M; Mayatepek, E; Okun, JG; Schulze-Bergkamen, A; Schulze-Bergkamen, H; Spiekerkötter, U; Zschocke, J1
Liu, A; Pasquali, M1
Balay, J; Ghoshal, AK; Soldin, SJ1
Dickenmann, M; Krähenbühl, S; Steiger, J; Vernez, L; Wenk, M1
Andresen, BS; Angel, L; Bennetts, B; Carpenter, K; Waddell, L; Wilcken, B; Wiley, V1
Roe, CR; Roe, DS; Struys, E; Sweetman, L; Vianey-Saban, C; Yang, BZ1
Adamovich, K; Arnecke, R; Decsi, T; Demmelmair, H; Koletzko, B; Lehner, F; Röschinger, W; Szász, M1
Bøersheim, E; Cree, MG; Sun, D; Wolfe, RR; Zhang, XJ1
Chace, DH; Kohli, R; Wainwright, MS; Whitington, PF1
Bennett, MJ; Fuda, F; Narayan, SB; Squires, RH1
de Sain, MG; Dorland, L; Engbers, HM; Eskes, PF; Visser, G1
Bodamer, OA; Hussein, K; Langhans, CD; Leonard, JV; Mayatepek, E; Morris, AA; Rating, D1
Böhm, M; Chrastina, R; Hansíková, H; Honzík, T; Martincová, O; Plavka, R; Zapadlo, M; Zeman, J1
Campbell, CD; Ficicioglu, C; Ganesh, J1
Beresford, MW; Davidson, JE; Pourfarzam, M; Turnbull, DM1
Inazu, M; Maehara, K; Matsumiya, T; Miyashita, K; Takeda, H; Tomoda, A1
Fouque, D; Guebre-Egziabher, F; Hadj-Aïssa, A; Holt, S; Hoppel, CL; Kopple, JD; Nakamura, K; Vianey-Saban, C1
Crnic, LR; Fricker, G; Goodman, SI; Harding, CO; Hoffmann, GF; Hörster, F; Koeller, DM; Kölker, S; Mahringer, A; Mühlhausen, C; Müller, I; Okun, JG; Sauer, SW1
Aledo, R; Casals, N; Champion, MP; Dalton, RN; Hegardt, FG; Mir, C; Pié, J; Turner, C1
Mattevi, A1
Chang, MH; Chen, HL; Chien, YH; Chiu, PC; Hwu, WL; Kobayashi, K; Lee, NC; Ni, YH; Saheki, T1
Bodamer, OA; Heinze, G; Holub, M; Mühl, A; Ratschmann, R; Sperl, W; Strnadová, KA; Tuschl, K1
Chan, CT; Fisher, L; Geary, DF; Hothi, DK1
Liebig, M; Mayatepek, E; Mueller, M; Schymik, I; Spiekerkoetter, U; Strauss, AW; Wanders, RJ; Wendel, U1
Calvani, M; Duran, M; Iannoni, E; Mancinelli, A1
Clark, S; Day, C; Henderson, MJ; Olpin, S; Patterson, A; Savill, G; Vijay, S; Walter, JH1
Hoppel, C; Minkler, P; Tserng, KY; Yang, S1
Duran, M; Huidekoper, HH; Schneider, J; Vaz, FM; Westphal, T; Wijburg, FA1
Azocar, M; Cano, F; Guerrero, JL; Marín, VB; Molina, M; Ratner, R1
Aguilar-Kitsu, A; Castillo-Romero, L; Ibarra-Cazares, P; Mendoza-Guevara, L; Morales-Nava, A; Perez Andrade, ME; Rodriguez-Leyva, F; Sanchez-Barbosa, L; Villasis-Keever, MA1
Anderson, BD; Freedenberg, D; Hahn, SH; Kramer, KA; Oglesbee, D1
Kamlekar, RK; Swamy, MJ1
Blobel, H; Gottschalk, J; Hoffmann, GF; Kölker, S; Köppel, S; Waterham, HR1
Chien, YH; Chu, KL; Huang, HP; Hwu, WL; Wang, SF; Wei, ML; Wu, ST1
Chen, YH; Chen, YT; Cheng, CF; Huang, CC; Hung, SI; Kao, HJ; Kikuchi, T; Millington, D; Wu, JY1
Choi, JH; Kim, GH; Park, SJ; Shin, YL; Yoo, HW; Yoon, HR1
Gerstner, T; Koenig, SA; Kohlmueller, D; Longin, E; Mehlem, P; Schulze, A; Teich, M; Treiss, I; Werner, T1
Bodamer, OA; Heinze, G; Holub, M; Lebl, J; Mascher, H; Mühl, A; Ratschmann, R; Stöckler-Ipsiroglu, S; Strnadová, KA; Votava, F; Waldhauser, F1
Ito, T; Kurono, Y; Maeda, Y; Sugiyama, N; Sumi, S; Suzuki, A; Togari, H; Ueta, A; Yokoi, K1
Endo, M; Hasegawa, Y; Kobayashi, H; Purevsuren, J; Yamaguchi, S1
Ahmad, A; Angle, B; Burton, B; Charrow, J; Ensenauer, R; Ficicioglu, CH; Hahn, SH; He, M; Keppen, LD; Majumder, N; Marsden, D; Matern, D; Oglesbee, D; Tortorelli, S; Vockley, J1
Dubois, J; Hulver, MW; Ilkayeva, O; Marchand, RM; Muoio, DM; Mynatt, RL; Power, RA; Zhang, JY1
Durkin, MS; Gleason, LA; Hoffman, G; Lindh, H; Rhead, W; van Calcar, SC; Vockley, G; Wolff, JA1
Ahn, EM; Cha, YS; Cho, SC; Lee, M1
Fok, TF; Ho, CS; Hui, J; Lam, CW; Law, LK; Ruiter, J; Tang, NL; Wanders, RJ1
Bennett, MJ1
Acott, PD; Al-Khalidi, M; Crocker, JF; Dooley, K; Geldenhuys, L; Lee, SH; Murphy, MG; O'Regan, P1
Chakrapani, A; Gray, G; Hendriksz, C; Macdonald, A; Pierre, G; Preece, MA1
Miwa, S; Takikawa, O1
Andersen, O; Andresen, BS; Beresford, MW; Cornelius, N; Dean, JC; Frerman, FE; Gregersen, N; Holme, E; Merinero, B; Miedzybrodzka, ZH; Morris, AA; Oldfors, A; Olpin, SE; Olsen, RK; Pourfarzam, M; Turnbull, DM1
Bodamer, OA; Hoffmann, GF; Lindner, M1
Hoppel, C; Minkler, P; Yang, S1
Hoppel, CL; Kerner, J; Lesnefsky, EJ; Minkler, PE1
Carrasco, P; Casals, N; Gratacós, E; Hegardt, FG; Jáuregui, O; Sierra, AY1
Garritson, B; Roe, CR; Roe, DS; Wallace, M1
Barshop, BA; Gangoiti, JA; Siuzdak, G; Wikoff, WR1
Adenier, H; Bourdin, B; Perrin, Y1
Ferrer, I; Merinero, B; Pérez-Cerdá, C; Ruiz-Sala, P; Ugarte, M; Vicente, Y1
Deng, L; Gong, QM; Kong, XF; La, DD; Li, HW; Peng, CH; Shen, BY; Shi, Q; Yu, YY; Zhang, XX; Zhu-Ge, CD1
Osorio, JH; Pourfarzam, M1
Chace, DH; Clark, RH; Flores, C; Kelleher, AS; Spitzer, AR; Steinbach, M; White, R1
Gao, XL; Gu, XF; Han, LS; Qiu, WJ; Wang, Y; Ye, J; Zhang, YJ1
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Akpan, IJ; Buehler, PW; Cendali, FI; D'Alessandro, A; Francis, RO; Gamboni, F; Hod, EA; Hudson, KE; Kahn, S; Katneni, U; Moriconi, C; Nemkov, T; Reisz, JA; Roh, D; Spitalnik, SL; Tanaka, K; Thangaraju, K; Thomas, T; Valk, JE; Wei, AZ; Zimring, JC1
Adam, T; Chrastina, P; Farolfi, M; Foltenová, H; Friedecký, D; Hanák, P; Honzík, T; Hrubá, E; Ješina, P; Kousal, B; Kožich, V; Pavlíková, M; Rücklová, K; Smolka, V; Vlášková, H; Zeman, J1
Bremer, AA; Cao, Y; Chen, L; Chen, Z; Gelaye, B; Grantz, KL; Hinkle, SN; Li, M; Mitro, SD; Rahman, ML; Song, Y; Tsai, MY; Weir, NL; Wu, J; Zhang, C; Zhu, Y1
He, X; Li, Q; Liu, H; Miao, J; Wan, K; Wang, D; Yuan, Z; Zhang, J; Zhu, J; Zou, L1
Dean, DA; Dorrestein, PC; Gautham, G; McCall, LI; McKerrow, JH; Siqueira-Neto, JL1
Cabrera, D; Falcón-Pérez, JM; Kollmus, H; Pilzner, C; Schughart, K; van Liempd, S1
Albillos, SM; Calvo, S; Cubo, E; Montero, O; Solano-Vila, B; Trejo, JM1
Li, Y; Lin, X; Liu, X; Ma, Y; Sun, L; Sun, Y; Zeng, R1
Ji, W; Tian, G; Wang, S; Wang, Y; Zhang, X1
An, ZN; Ge, MH; Jiang, DW; Liu, C; Shen, XL; Wei, DZ; Xing, XJ; Zheng, DM1
Dambrova, M; Goldins, NR; Grinberga, S; Groma, V; Kuka, J; Liepinsh, E; Makrecka-Kuka, M; Plaas, M; Sevostjanovs, E; Stelfa, G; Svalbe, B; Vilks, K; Vilskersts, R1
Ding, D; Gong, P; Li, W; Wang, N; Wang, X; Wang, Z; Zhang, X1
Drake, RR; Louey, S; Thornburg, KL1
Agarwal, A; Brantley, MA; Haines, JL; Jones, DP; Ma, C; Mitchell, SL; Pericak-Vance, MA; Scott, WK; Uppal, K1
Almanza-Aguilera, E; Alonso-Gómez, Á; Arós, F; Clish, CB; Corella, D; Dennis, C; Estruch, R; Fiol, M; Gómez-Gracia, E; Guasch-Ferré, M; Hernández-Alonso, P; Hu, FB; Lapetra, J; Li, J; Liang, L; Martínez-González, MÁ; Razquin, C; Ros, E; Ruiz-Canela, M; Salas-Salvadó, J; Serra-Majem, L; Toledo, E; Wittenbecher, C1
Abily-Donval, L; Bekri, S; Goldenberg, A; Marret, S; Salomons, GS; Snanoudj, S; Sudrié-Arnaud, B; Tebani, A; Torre, S1
Griffin, C; Luna, C; Miller, MJ1
Borradaile, N; Bureau, Y; Dunlop, K; Hadway, J; Lee, TY; Nygard, K; Regnault, TRH; Richardson, BS; Sarr, O; Stachura, N; Thompson, JA; Zhao, L1
Adebayo, AS; Anthony, B; Aujla, H; Dott, W; Eagle-Hemming, B; Griffin, JL; Gulston, M; Joel-David, L; Kumar, T; Lai, FY; Murgia, A; Murphy, GJ; Roman, M; Sheikh, S; Woźniak, MJ; Yusoff, S; Zakkar, M1
Deda, O; Gika, H; Karagiannidis, E; Meikopoulos, T; Sianos, G; Theodoridis, G1
An, Z; Jiang, D; Liu, C; Wei, D; Xing, X; Zheng, D1
Aimo, A; Franco, AD; Morfino, P1
Cao, Y; Jiang, Y; Shi, H; Song, W; Wang, Y; Zhang, B; Zhang, Z; Zhu, L1
Boemer, F; Cavalier, E; Misset, B; Rousseau, AF; Schmitz, S1
Begou, O; Deda, O; Gika, H; Karagiannidis, E; Meikopoulos, T; Mouskeftara, T; Panteris, E; Papazoglou, AS; Sianos, G; Theodoridis, G1
Dang, Y; Dong, J; Fan, G; Meng, L; Shen, J; Shi, H; Shi, J; Wang, D; Wu, W; Xi, H; Yang, R; Yu, P1
Fang, ZZ; Gao, XQ; Li, X; Liu, X; Wang, WY1
Arnold, M; Dehkordi, SM; Dunlop, BW; Kaddurah-Daouk, R; Kastenmüller, G; Krishnan, RR; Milaneschi, Y; Penninx, BWJH; Rush, AJ1
Fan, S; Jia, X; OuYang, B; Wang, S; Wu, B; Zhang, N1
Alavi-Moghadam, S; Arjmand, B; Dehghanbanadaki, H; Dilmaghani-Marand, A; Farzadfar, F; Ghodssi-Ghassemabadi, R; Hosseinkhani, S; Larijani, B; Mohammadi Fateh, S; Najjar, N; Nasli-Esfahani, E; Razi, F1
Aaron, L; Borkowsky, W; Hobbs, C; Kirmse, B; Montepiedra, G; Ryckman, KK; Smith, CJ; Summar, M; Van Dyke, R; Williams, PL; Yu, C1
Attwood, MM; Dambrova, M; Guo, AC; Kuka, J; Liepinsh, E; Makrecka-Kuka, M; Nordberg, D; Oler, E; Schiöth, HB; Sen, ZD; Smesny, S; Tian, S; Vilskersts, R; Wishart, DS; Zheng, J1
Amiano, P; Ardanaz, E; Chajes, V; Gunter, MJ; Huerta, JM; Hultdin, J; Huybrechts, I; Jakszyn, P; Johnson, T; Katzke, V; Keski-Rahkonen, P; Krogh, V; Pokharel, P; Redondo-Sánchez, D; Rothwell, JA; Sacerdote, C; Sandanger, TM; Santucci de Magistris, M; Scalbert, A; Schmidt, JA; Schulze, MB; Tjønneland, A; Tumino, R; Viallon, V; Wedekind, R; Weiderpass, E; Winkvist, A1
Calvano, CD; Cataldi, TRI; Console, L; Giangregorio, N; Incampo, G; Indiveri, C; Marino, T; Prejanò, M; Russo, N; Scalise, M; Tonazzi, A1
Honda, M; Honda, Y; Miyagawa, T; Shigematsu, Y; Shimada, M; Tokunaga, K1
Calderón-Santiago, M; Luque-Córdoba, D; Priego-Capote, F1
Asadi, M; Bandarian, F; Dehghanbanadaki, H; Hosseinkhani, S; Najjar, N; Pasalar, P; Razi, F; Salari, P; Shirani, S1
Altay, N; Binici, O; Büyükfırat, E; Duran, E; Gönel, A; Karahan, MA; Koyuncu, I1
Feng, T; Kenéz, Á; Mann, S; Nydam, DV; Overton, TR; Wakshlag, JJ; Xu, W; Yepes, FL1
Emamgholipour, S; Hosseinkhani, S; Khalagi, K; Larijani, B; Najjar, N; Pasalar, P; Razi, F; Salari, P; Shirani, S1
Khatami, S; Lotfi, J; Rabbani, A; Rasaee, MJ; Taghikhani, M1
Franke, L; Peters, FT; Wissenbach, DK1
Chammas, R; Chuffa, LGA; Junior, RP; Reiter, RJ; Simão, VA; Sonehara, NM; Zuccari, DAPC1
Jacobs, A; Jansen Van Vuren, E; Kruger, R; Louw, R; Mels, C; Strauss-Kruger, M1
Chen, D; Chen, YM; Fu, Y; Gou, W; Jiang, Z; Liu, CY; Wu, YY; Xie, K; Yan, Y; Yang, Y; Zheng, JS; Zhu, HL1
Brumarová, R; Dobešová, D; Friedecký, D; Jahn, P; Kopecká, A; Tóthová, K1
De Grassi, A; Giangregorio, N; Incampo, G; Indiveri, C; Pierri, CL; Tonazzi, A; Tragni, V1
Clinton Frazee, C; Garg, U; Heese, B; Scott, D1
Brose, SA; Hobert, JA; Pasquali, M1
Baird, S; Frazee, CC; Garg, U1
Canizales-Quinteros, S; Flores, YN; Ibarra-González, I; León-Reyes, G; Palacios-González, B; Rivera-Paredez, B; Salmerón, J; Vela-Amieva, M; Velázquez-Cruz, R1
Fu, Z; He, B; Hu, L; Jin, C; Jin, Y; Wang, C; Wang, X1
Abali, S; Albayrak, E; Basgoze, S; Beken, S; Bulum, B; Demirelce, O; Erek, E; Guvenc, O; Odemis, E; Saral, NY; Serteser, M; Toygar, AK; Yalcin, G1
Li, D; Lu, Y; Wei, L; Yan, L; Yang, K; Yang, X; Yuan, X; Zhao, F1
Aktuğlu Zeybek, AÇ; Barut, K; Cansever, MŞ; Kasapçopur, Ö; Kıykım, E; Zübarioğlu, T1
Attia, SL; Helsley, RN; Inci, MK; Park, SH; Softic, S1
Cai, R; Chang, R; Chen, D; Huang, L; Jiang, Y; Ning, H; Pan, L; Tan, J; Wei, B; Wei, J; Yang, J; Yin, B; Yuan, D; Zeng, T; Zheng, M1
Chen, Y; Cheng, X; Li, M; Liang, Z; Tan, X; Wang, W; Weng, X; Wu, M; Wu, X; Zhang, Z; Zhu, P; Zhu, R1
Akbay, Hİ; Akmeşe, Ş; Alp, HH; Binici, I; Çelik, H; Gonel, A; Huyut, Z; Karsen, H; Koyuncu, I1
Burton, GJ; El-Bacha, T; Ferreira, CS; Goes, VA; Griffin, JL; Guimarães, DAB; Lai, C; Murgia, A; Pinto, GDA; Ranquine, LG; Reis, DL; Struchiner, CJ; Torres, AG1
Gika, H; Kotali, A; Theodoridis, K1
Hotwani, A; Ilyas, M; Jafri, L; Jehan, F; Khalid, J; Khan, AH; Majid, H; Nisar, I1
Abellan-Borja, A; Barboza-Aranda, LJ; Carrera-Treviño, R; Delgado-Enciso, I; Gomez-Govea, MA; Gonzalez-Rojas, JI; Guzman-Velasco, A; Martinez-de-Villareal, LE; Martinez-Fierro, ML; Rodriguez-Sanchez, IP; Ruiz-Ayma, G; Villanueva-Segura, OK; Zapata-Morin, PA1
Arjmand, B; Asadi, M; Dehghanbanadaki, H; Dilmaghani-Marand, A; Emamgholipour, S; Ghasemi, E; Ghodssi-Ghassemabadi, R; Hosseinkhani, S; Khoshniat, M; Larijani, B; Najjar, N; Panahi, N; Razi, F; Rezaei, N; Taghizadeh, H1
Kim, YA; Kwon, SH; Lee, Y; Park, UC; Seo, JH; Yoo, HJ; Yoon, CK; Yu, HG1
Goto, K; Hiramatsu, M; Imai, K; Korematsu, S; Uchiyama, SI; Uemura, A; Wasada, R1
Dambrova, M; Grandane, A; Jaudzems, K; Liepinsh, E; Pudnika-Okinčica, L; Sipola, A; Videja, M; Vilks, K; Zelencova-Gopejenko, D1
Artiushin, G; Li, F; Sehgal, A1
Li, CS; Lu, ZQ; Wang, GX; Xiao, HL; Xie, MR; Zhang, HY1
Elgstøen, KBP; Hiller, JK; Moe, MC; Petrovski, G; Rootwelt, H; Sandås, EM; Utheim, TP; Østeby Vassli, A1
Dawid, C; Dunkel, A; Ensenauer, R; Hofmann, TF; Kleigrewe, K; Mittermeier-Klessinger, VK; Wudy, SI1
Miller, M; Morrissey, M; Rastogi, D; Rastogi, S; Sasenick, J1
Console, L; Giangregorio, N; Indiveri, C; Scalise, M; Tonazzi, A1
Luo, H; Zhu, Z1
Davies, A; Hazen, SL; Klingenberg, R; Laaksonen, R; Li, XS; Lüscher, TF; Mach, F; Matter, CM; Muller, O; Obeid, S; Räber, L; Wang, Z; Wenzl, FA; Winzap, P1
Bie, X; Chen, G; Chen, L; Hao, Y; Liu, H; Liu, X; Ma, X; Shi, X; Yan, W; Yang, F; Yang, J; Yang, X; Zhang, D; Zhang, K; Zhao, D; Zheng, H; Zhu, Z1
Aitken-Buck, HM; Jones, PP; Khaing, EP; Lamberts, RR1
Domschke, K; Endres, D; Maier, S; Menke, M; Nickel, K; Runge, K; Schumann, A; Tebartz van Elst, L; Tucci, S1
Hayashida, S; Isobe, N; Shiraishi, W; Tajima, G; Tateishi, T; Tsumura, M1
Abi-Rached, ZH; Jennings, EA; Jones, DE; Ryan, RO1
Borum, PR; Broquist, HP1
Dewhurst, SA; Weinreich, D1
Lees, GJ; McCaman, MW; McCaman, RE1
Fava, M; Mischoulon, D; Papakostas, GI; Sarris, J; Vitolo, O1
Aithal, GP; Alvarez-Alvarez, I; Andrade, RJ; Arikan, C; Atallah, E; Lucena, MI; Medina-Caliz, I; Niu, H1

Reviews

52 review(s) available for carnitine and s-adenosylmethionine

ArticleYear
[Fibromyalgia syndrome].
    Nihon rinsho. Japanese journal of clinical medicine, 1999, Volume: 57, Issue:2

    Topics: Biomarkers; Carnitine; Diagnosis, Differential; Fatigue; Fatigue Syndrome, Chronic; Fibromyalgia; Humans; Mood Disorders; Pain; Syndrome

1999
Supplemental carnitine and exercise.
    The American journal of clinical nutrition, 2000, Volume: 72, Issue:2 Suppl

    Topics: Carnitine; Coenzyme A; Dietary Supplements; Exercise; Fatty Acids; Female; Humans; Male; Muscle Contraction; Muscle, Skeletal; Oxygen Consumption

2000
[Chronic fatigue syndrome].
    Ryoikibetsu shokogun shirizu, 2000, Issue:32

    Topics: Carnitine; Diagnosis, Differential; Endocrine System Diseases; Fatigue Syndrome, Chronic; Humans; Immune System Diseases; Prognosis; Stress, Physiological; Virus Diseases

2000
[Mitochondrial carnitine acylcarnitine translocase].
    Nihon rinsho. Japanese journal of clinical medicine, 2002, Volume: 60 Suppl 4

    Topics: Carnitine; Carnitine Acyltransferases; Energy Metabolism; Fatty Acids; Humans; Mitochondria; Oxidation-Reduction

2002
Application of tandem mass spectrometry to biochemical genetics and newborn screening.
    Clinica chimica acta; international journal of clinical chemistry, 2002, Volume: 322, Issue:1-2

    Topics: Amino Acids; Carnitine; Genetic Testing; Humans; Infant, Newborn; Molecular Biology; Neonatal Screening; Peroxisomal Disorders; Spectrometry, Mass, Electrospray Ionization

2002
Prospective diagnosis of 2-methylbutyryl-CoA dehydrogenase deficiency in the Hmong population by newborn screening using tandem mass spectrometry.
    Pediatrics, 2003, Volume: 112, Issue:1 Pt 1

    Topics: Amino Acid Metabolism, Inborn Errors; Carnitine; Diagnosis, Differential; Diet, Protein-Restricted; DNA Mutational Analysis; Ethnicity; Exons; Female; Genetic Testing; Humans; Infant, Newborn; Intellectual Disability; Isoleucine; Male; Mass Spectrometry; Neonatal Screening; Oxidoreductases Acting on CH-CH Group Donors; Sequence Deletion; Treatment Outcome

2003
Use of tandem mass spectrometry for multianalyte screening of dried blood specimens from newborns.
    Clinical chemistry, 2003, Volume: 49, Issue:11

    Topics: Amino Acid Metabolism, Inborn Errors; Amino Acids; Blood Specimen Collection; Carnitine; Hematologic Tests; Humans; Infant, Newborn; Lipid Metabolism, Inborn Errors; Mass Spectrometry

2003
L-carnitine and its possible role in red cell and platelet storage.
    Transfusion medicine reviews, 2004, Volume: 18, Issue:1

    Topics: Blood Platelets; Blood Preservation; Carnitine; Erythrocytes; Fatty Acids; Humans; Hydrogen-Ion Concentration; Intracellular Membranes; Mitochondria; Models, Chemical; Oxygen; Platelet Transfusion; Specimen Handling; Temperature; Time Factors

2004
Carnitine-acylcarnitine translocase deficiency: identification of a novel molecular defect in a Bedouin patient.
    Journal of inherited metabolic disease, 2004, Volume: 27, Issue:2

    Topics: Arabs; Carnitine; Carnitine Acyltransferases; Fatal Outcome; Humans; Infant, Newborn; Israel; Male; Metabolism, Inborn Errors; Mutation, Missense; Oxidation-Reduction

2004
Carnitine and peripheral arterial disease.
    Annals of the New York Academy of Sciences, 2004, Volume: 1033

    Topics: Arterial Occlusive Diseases; Carnitine; Humans; Intermittent Claudication; Muscle, Skeletal; Oxygen Consumption

2004
Assays of fatty acid beta-oxidation activity.
    Methods in cell biology, 2007, Volume: 80

    Topics: Acids; Biological Transport, Active; Carnitine; Fatty Acids; Glycine; Humans; Lipid Metabolism; Mitochondria; Models, Biological; Oxidation-Reduction

2007
[Chronic fatigue syndrome and neurotransmitters].
    Nihon rinsho. Japanese journal of clinical medicine, 2007, Volume: 65, Issue:6

    Topics: Animals; Carnitine; Fatigue Syndrome, Chronic; Glutamates; Humans; Membrane Proteins; Neurotransmitter Agents; Organic Cation Transport Proteins; Serotonin; Serotonin Plasma Membrane Transport Proteins; Solute Carrier Family 22 Member 5; Synaptic Transmission

2007
[Determination of normal acylcarnitine levels in a healthy pediatric population as a diagnostic tool in inherited errors of mitochondrial fatty acid beta-oxidation].
    Anales de pediatria (Barcelona, Spain : 2003), 2007, Volume: 67, Issue:6

    Topics: Adolescent; Carnitine; Child; Child, Preschool; Fatty Acids; Female; Humans; Infant; Lipid Metabolism, Inborn Errors; Male; Mitochondrial Diseases; Oxidation-Reduction; Reference Values

2007
Is oral L-acyl-carnitine an effective therapy for hepatic encephalopathy? Review of the literature.
    Digestive diseases and sciences, 2008, Volume: 53, Issue:9

    Topics: Administration, Oral; Carnitine; Hepatic Encephalopathy; Humans; Randomized Controlled Trials as Topic; Treatment Outcome

2008
Interstitial deletion of 1p22.2p31.1 and medium-chain acyl-CoA dehydrogenase deficiency in a patient with global developmental delay.
    American journal of medical genetics. Part A, 2008, Jun-15, Volume: 146A, Issue:12

    Topics: Acyl-CoA Dehydrogenase; Carnitine; Child; Chromosome Deletion; Chromosomes, Human, Pair 1; Dicarboxylic Acids; DNA Mutational Analysis; Exons; Facies; Female; Humans; In Situ Hybridization, Fluorescence; Karyotyping; Mutation, Missense; Oligonucleotide Array Sequence Analysis; Psychomotor Disorders; Seizures

2008
Carnitine, mitochondrial function and therapy.
    Advanced drug delivery reviews, 2009, Nov-30, Volume: 61, Issue:14

    Topics: Animals; Carnitine; Carnitine Acyltransferases; Heart Diseases; Humans; Mitochondria

2009
Acylcarnitines: role in brain.
    Progress in lipid research, 2010, Volume: 49, Issue:1

    Topics: Acetylcarnitine; Animals; Antioxidants; Brain; Carnitine; Energy Metabolism; Fatty Acids; Nerve Growth Factor; Nervous System Diseases; Neuroprotective Agents

2010
Muscle insulin resistance: assault by lipids, cytokines and local macrophages.
    Current opinion in clinical nutrition and metabolic care, 2010, Volume: 13, Issue:4

    Topics: Adipose Tissue; Animals; Carnitine; Cytokines; Dietary Fats; Humans; Insulin; Insulin Resistance; Lipid Metabolism; Macrophages; Muscle, Skeletal; Phosphotransferases; Reactive Oxygen Species; Triglycerides

2010
LC-MS/MS progress in newborn screening.
    Clinical biochemistry, 2011, Volume: 44, Issue:1

    Topics: Carnitine; Chromatography, Liquid; Humans; Infant, Newborn; Lysosomal Storage Diseases; Neonatal Screening; Tandem Mass Spectrometry

2011
How to use acylcarnitine profiles to help diagnose inborn errors of metabolism.
    Archives of disease in childhood. Education and practice edition, 2010, Volume: 95, Issue:5

    Topics: Carnitine; Chemistry, Clinical; Child; Humans; Metabolism, Inborn Errors; Pediatrics

2010
Profiling of acylcarnitines and sterols from dried blood or plasma spot by atmospheric pressure thermal desorption chemical ionization (APTDCI) tandem mass spectrometry.
    Biochimica et biophysica acta, 2011, Volume: 1811, Issue:11

    Topics: Atmospheric Pressure; Carnitine; Dried Blood Spot Testing; Humans; Sterols; Tandem Mass Spectrometry; Temperature

2011
Carnitine and acylcarnitines: pharmacokinetic, pharmacological and clinical aspects.
    Clinical pharmacokinetics, 2012, Sep-01, Volume: 51, Issue:9

    Topics: Animals; Carnitine; Homeostasis; Humans

2012
Application of mass spectrometry in newborn screening: about both small molecular diseases and lysosomal storage diseases.
    Topics in current chemistry, 2014, Volume: 336

    Topics: Amino Acids; Carnitine; Humans; Infant, Newborn; Lysosomal Storage Diseases; Metabolism, Inborn Errors; Neonatal Screening; Tandem Mass Spectrometry

2014
Universal screening for inherited metabolic diseases in the neonate (and the fetus).
    The journal of maternal-fetal & neonatal medicine : the official journal of the European Association of Perinatal Medicine, the Federation of Asia and Oceania Perinatal Societies, the International Society of Perinatal Obstetricians, 2012, Volume: 25, Issue:Suppl 5

    Topics: Acyl-CoA Dehydrogenase; Amino Acids; Carnitine; DNA; DNA Mutational Analysis; Female; Gestational Age; Humans; Infant, Newborn; Metabolism, Inborn Errors; Neonatal Screening; Paper; Phenylketonurias; Pregnancy; Prenatal Diagnosis; Tandem Mass Spectrometry

2012
Acylcarnitines: reflecting or inflicting insulin resistance?
    Diabetes, 2013, Volume: 62, Issue:1

    Topics: Animals; Carnitine; Citric Acid Cycle; Fatty Acids; Humans; Insulin Resistance; Lipid Metabolism; Oxidation-Reduction

2013
Separation of carnitine and acylcarnitines in biological samples: a review.
    Biomedical chromatography : BMC, 2013, Volume: 27, Issue:10

    Topics: Animals; Carnitine; Chromatography, Liquid; Electrophoresis, Capillary; Gas Chromatography-Mass Spectrometry; Humans; Liquid-Liquid Extraction; Solid Phase Extraction

2013
Metabolomic biomarkers for obesity in humans: a short review.
    Annals of nutrition & metabolism, 2014, Volume: 64, Issue:3-4

    Topics: Amino Acids, Branched-Chain; Biomarkers; Carnitine; Case-Control Studies; Cross-Sectional Studies; Humans; Metabolomics; Obesity

2014
Risks and benefits of carnitine supplementation in diabetes.
    Experimental and clinical endocrinology & diabetes : official journal, German Society of Endocrinology [and] German Diabetes Association, 2015, Volume: 123, Issue:2

    Topics: Carnitine; Diabetes Mellitus; Dietary Supplements; Humans; Risk

2015
Nature and nurture in atherosclerosis: The roles of acylcarnitine and cell membrane-fatty acid intermediates.
    Vascular pharmacology, 2016, Volume: 78

    Topics: Animals; Atherosclerosis; Carnitine; Cell Membrane; Dietary Fats; Disease Progression; Erythrocytes; Fatty Acids; Humans; Lipids; Macrophages

2016
Acylcarnitines--old actors auditioning for new roles in metabolic physiology.
    Nature reviews. Endocrinology, 2015, Volume: 11, Issue:10

    Topics: Carnitine; Humans; Lipid Metabolism; Lipid Metabolism, Inborn Errors; Metabolic Diseases; Metabolic Networks and Pathways; Metabolism

2015
Metabolomics in diabetes, a review.
    Annals of medicine, 2016, Volume: 48, Issue:1-2

    Topics: Amino Acids, Branched-Chain; Animals; Biomarkers; Carnitine; Diabetes Mellitus, Type 2; Humans; Insulin Resistance; Metabolomics; Obesity; Predictive Value of Tests

2016
Inborn Errors of Metabolism That Cause Sudden Infant Death: A Systematic Review with Implications for Population Neonatal Screening Programmes.
    Neonatology, 2016, Volume: 109, Issue:4

    Topics: Amino Acids; Autopsy; Carnitine; Humans; Infant; Infant, Newborn; Metabolism, Inborn Errors; Neonatal Screening; Reye Syndrome; Sudden Infant Death

2016
Measurement of β-oxidation capacity of biological samples by respirometry: a review of principles and substrates.
    American journal of physiology. Endocrinology and metabolism, 2016, 05-01, Volume: 310, Issue:9

    Topics: Acyl Coenzyme A; Carnitine; Coenzyme A; Electrodes; Fatty Acids; Feedback, Physiological; Humans; Malates; Mitochondria; Oxidation-Reduction; Oxidative Phosphorylation; Oxygen Consumption; Palmitoylcarnitine

2016
Association of Plasma Small-Molecule Intermediate Metabolites With Age and Body Mass Index Across Six Diverse Study Populations.
    The journals of gerontology. Series A, Biological sciences and medical sciences, 2016, Volume: 71, Issue:11

    Topics: Aged; Aging; Amino Acids; Body Mass Index; Carnitine; Female; Humans; Male; Metabolomics; Obesity; Plasma

2016
Using Metabolomic Profiles as Biomarkers for Insulin Resistance in Childhood Obesity: A Systematic Review.
    Journal of diabetes research, 2016, Volume: 2016

    Topics: Amino Acids, Aromatic; Amino Acids, Branched-Chain; Biomarkers; Carnitine; Child; Humans; Insulin Resistance; Metabolomics; Pediatric Obesity

2016
Metabolomics for clinical use and research in chronic kidney disease.
    Nature reviews. Nephrology, 2017, Volume: 13, Issue:5

    Topics: Arginine; Biomarkers; Biomedical Research; Carnitine; Diabetes Mellitus; Humans; Lipid Metabolism; Metabolomics; Oxidative Stress; Renal Insufficiency, Chronic

2017
Do diabetes and obesity affect the metabolic response to exercise?
    Current opinion in clinical nutrition and metabolic care, 2017, Volume: 20, Issue:4

    Topics: AMP-Activated Protein Kinases; Blood Glucose; Carnitine; Diabetes Mellitus, Type 2; Exercise; Fatty Acids; Glucose; Glycogen; Humans; Liver; Muscle, Skeletal; Obesity; Oxidation-Reduction; Peroxisome Proliferator-Activated Receptor Gamma Coactivator 1-alpha; Signal Transduction

2017
Biotin: From Nutrition to Therapeutics.
    The Journal of nutrition, 2017, Volume: 147, Issue:8

    Topics: Animals; Biotin; Biotinylation; Carnitine; Female; Gene Expression; Hormones; Humans; Multiple Sclerosis; Nutritional Status; Pregnancy; Pregnancy Complications; Streptavidin; Vitamin B Complex

2017
Loading and firing the brown adipocyte.
    Adipocyte, 2018, 01-02, Volume: 7, Issue:1

    Topics: Adipocytes, Brown; Adipose Tissue, Brown; Adipose Tissue, White; Animals; Carnitine; Fatty Acids; Glucose; Humans; Uncoupling Protein 1

2018
Biomarkers for mitochondrial energy metabolism diseases.
    Essays in biochemistry, 2018, 07-20, Volume: 62, Issue:3

    Topics: Amino Acids; Biomarkers; Carnitine; Creatine Kinase; Energy Metabolism; Fibroblast Growth Factors; Growth Differentiation Factor 15; Humans; Lactic Acid; Mitochondrial Diseases; Oxidative Phosphorylation; Pyruvic Acid

2018
Physiology of L-carnitine in plants in light of the knowledge in animals and microorganisms.
    Plant science : an international journal of experimental plant biology, 2018, Volume: 274

    Topics: Animals; Carnitine; Carnitine Acyltransferases; Fatty Acids; Lipid Metabolism; Mitochondria; Plants

2018
Autologous cord blood in children with cerebral palsy: a review.
    International journal of molecular sciences, 2019, May-16, Volume: 20, Issue:10

    Topics: 3-Hydroxybutyric Acid; Amino Acids; Animals; Brain Diseases; Brain Injuries; Carnitine; Cerebral Palsy; Child; Cytokines; Databases, Factual; Fetal Blood; Glycerol; Humans; Hypoxia; Hypoxia-Ischemia, Brain; Intercellular Signaling Peptides and Proteins; Phosphatidylcholines; Phosphorylcholine; Stem Cells; Succinic Acid

2019
Carnitine in Human Muscle Bioenergetics: Can Carnitine Supplementation Improve Physical Exercise?
    Molecules (Basel, Switzerland), 2020, Jan-01, Volume: 25, Issue:1

    Topics: Carnitine; Carnitine O-Palmitoyltransferase; Dietary Supplements; Energy Metabolism; Exercise; Fatty Acids; Humans; Methylamines; Mitochondria; Muscle, Skeletal; Oxidation-Reduction

2020
Review: Lipid biology in the periparturient dairy cow: contemporary perspectives.
    Animal : an international journal of animal bioscience, 2020, Volume: 14, Issue:S1

    Topics: Animals; Carnitine; Cattle; Ceramides; Energy Metabolism; Fatty Acids; Female; Glucose; Glycerophospholipids; Insulin; Insulin Resistance; Lactation; Lipid Metabolism; Lipolysis; Liver; Milk; Mitochondria; Signal Transduction; Sphingolipids; Triglycerides

2020
Metabolic interactions between peroxisomes and mitochondria with a special focus on acylcarnitine metabolism.
    Biochimica et biophysica acta. Molecular basis of disease, 2020, 05-01, Volume: 1866, Issue:5

    Topics: Carnitine; Carnitine Acyltransferases; Fatty Acids; Mitochondria; Peroxisomes

2020
Genomics-based identification of a potential causal role for acylcarnitine metabolism in depression.
    Journal of affective disorders, 2022, 06-15, Volume: 307

    Topics: Carnitine; Causality; Depression; Genomics; Humans

2022
Acylcarnitines: Nomenclature, Biomarkers, Therapeutic Potential, Drug Targets, and Clinical Trials.
    Pharmacological reviews, 2022, Volume: 74, Issue:3

    Topics: Biomarkers; Carnitine; Fatty Acids; Humans; Insulin Resistance

2022
Fructose impairs fat oxidation: Implications for the mechanism of western diet-induced NAFLD.
    The Journal of nutritional biochemistry, 2023, Volume: 114

    Topics: Animals; Carnitine; Diet, High-Fat; Diet, Western; Dietary Fats; Fructose; Humans; Liver; Non-alcoholic Fatty Liver Disease; PPAR alpha

2023
Acylcarnitines in Ophthalmology: Promising Emerging Biomarkers.
    International journal of molecular sciences, 2022, Dec-19, Volume: 23, Issue:24

    Topics: Biomarkers; Carnitine; Glaucoma, Open-Angle; Humans; Infant, Newborn; Ophthalmology; Vitreous Body

2022
Serum acylcarnitines levels as a potential predictor for gestational diabetes: a systematic review and meta-analysis.
    Frontiers in public health, 2023, Volume: 11

    Topics: Carnitine; Child; Diabetes, Gestational; Female; Humans; Mothers; Pregnancy

2023
3-Methylglutarylcarnitine: A biomarker of mitochondrial dysfunction.
    Clinica chimica acta; international journal of clinical chemistry, 2023, Nov-01, Volume: 551

    Topics: Biomarkers; Carnitine; Humans; Leucine; Mitochondria

2023
Therapeutic Management of Idiosyncratic Drug-Induced Liver Injury and Acetaminophen Hepatotoxicity in the Paediatric Population: A Systematic Review.
    Drug safety, 2022, Volume: 45, Issue:11

    Topics: Acetaminophen; Acetylcysteine; Adolescent; Adrenal Cortex Hormones; Adult; Carnitine; Chemical and Drug Induced Liver Injury; Child; Drug-Related Side Effects and Adverse Reactions; Glutathione; Glycyrrhizic Acid; Humans; Infant, Newborn; Liver; Liver Failure, Acute; Retrospective Studies; S-Adenosylmethionine; Ursodeoxycholic Acid

2022

Trials

69 trial(s) available for carnitine and s-adenosylmethionine

ArticleYear
L-carnitine supplementation in patients with cystic acne on isotretinoin therapy.
    Journal of the European Academy of Dermatology and Venereology : JEADV, 1999, Volume: 13, Issue:3

    Topics: Acne Vulgaris; Adult; Alanine Transaminase; Alkaline Phosphatase; Aspartate Aminotransferases; Carnitine; Case-Control Studies; Cholesterol; Female; Follow-Up Studies; Humans; Isotretinoin; Keratolytic Agents; Liver; Male; Muscle Weakness; Muscular Diseases; Pain; Placebos; Triglycerides

1999
Intravenous L-carnitine increases plasma carnitine, reduces fatigue, and may preserve exercise capacity in hemodialysis patients.
    American journal of kidney diseases : the official journal of the National Kidney Foundation, 2001, Volume: 37, Issue:5

    Topics: Adult; Aged; Carbon Dioxide; Carnitine; Double-Blind Method; Exercise Test; Exercise Tolerance; Fatigue; Female; Humans; Injections, Intravenous; Kidney Failure, Chronic; Kidney Transplantation; Male; Middle Aged; Models, Biological; Oxygen Consumption; Quality of Life; Renal Dialysis

2001
Metabolic effects in neonates receiving intravenous medium-chain triglycerides.
    Acta paediatrica (Oslo, Norway : 1992), 2002, Volume: 91, Issue:2

    Topics: Carnitine; Double-Blind Method; Fat Emulsions, Intravenous; Fatty Acids, Nonesterified; Female; Humans; Infant, Newborn; Male; Muscle, Skeletal; Parenteral Nutrition, Total; Triglycerides

2002
Carnitine metabolism in diabetes mellitus.
    Journal of pediatric endocrinology & metabolism : JPEM, 2002, Volume: 15, Issue:6

    Topics: Adolescent; Adult; Carnitine; Child; Child, Preschool; Diabetes Mellitus, Type 1; Diabetic Angiopathies; Fatty Acids; Female; Glycated Hemoglobin; Humans; Insulin; Lipids; Lipoprotein(a); Male; Risk Factors

2002
Optimal dietary therapy of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency.
    Molecular genetics and metabolism, 2003, Volume: 79, Issue:2

    Topics: 3-Hydroxyacyl CoA Dehydrogenases; Carnitine; Child; Child, Preschool; Diet Therapy; Energy Intake; Fatty Acids; Female; Humans; Infant; Lipid Metabolism, Inborn Errors; Long-Chain-3-Hydroxyacyl-CoA Dehydrogenase; Male; Minerals; Multienzyme Complexes; Treatment Outcome; Vitamins

2003
L-carnitine and exercise tolerance in medium-chain acyl-coenzyme A dehydrogenase (MCAD) deficiency: a pilot study.
    Journal of inherited metabolic disease, 2005, Volume: 28, Issue:2

    Topics: 3-Hydroxybutyric Acid; Acids; Acyl-CoA Dehydrogenase; Adolescent; Adult; Anaerobic Threshold; Blood Glucose; Carnitine; Child; Exercise; Exercise Test; Fatty Acids, Nonesterified; Female; Humans; Lactic Acid; Life Style; Male; Metabolism, Inborn Errors; Pilot Projects

2005
Metabolic effects of intravenous LCT or MCT/LCT lipid emulsions in preterm infants.
    Journal of lipid research, 2006, Volume: 47, Issue:2

    Topics: 3-Hydroxybutyric Acid; alpha-Linolenic Acid; alpha-Tocopherol; Body Weight; Carbon Isotopes; Carnitine; Cholesterol; Double-Blind Method; Fat Emulsions, Intravenous; Fatty Acids; Fatty Acids, Essential; Fatty Acids, Nonesterified; Fatty Acids, Unsaturated; Female; gamma-Tocopherol; Humans; Infant, Newborn; Infant, Premature; Lipid Metabolism; Male; Phospholipids; Triglycerides

2006
Demographic and nutritional factors associated with prolonged cholestatic jaundice in the premature infant.
    Journal of perinatology : official journal of the California Perinatal Association, 2008, Volume: 28, Issue:2

    Topics: Amino Acids; Birth Weight; Carnitine; Dietary Supplements; Gestational Age; Glucocorticoids; Humans; Infant; Infant, Newborn; Infant, Premature; Jaundice, Obstructive; Logistic Models; Parenteral Nutrition

2008
The risk of asymptomatic hyperammonemia in children with idiopathic epilepsy treated with valproate: relationship to blood carnitine status.
    Epilepsy research, 2009, Volume: 86, Issue:1

    Topics: Adolescent; Ammonia; Anticonvulsants; Carnitine; Child; Epilepsy; Female; Humans; Hyperammonemia; Male; Prospective Studies; Valproic Acid; Vitamin B Complex

2009
Effect of triheptanoin on muscle metabolism during submaximal exercise in horses.
    American journal of veterinary research, 2009, Volume: 70, Issue:8

    Topics: Analysis of Variance; Animals; Blood Glucose; Carbohydrate Metabolism; Carnitine; Corn Oil; Cross-Over Studies; Horses; Insulin; Lipid Metabolism; Muscle, Skeletal; Physical Conditioning, Animal; Random Allocation; Triglycerides

2009
Relationship between carnitine, fatty acids and insulin resistance.
    Gynakologisch-geburtshilfliche Rundschau, 2009, Volume: 49, Issue:4

    Topics: Carnitine; Carnitine O-Acetyltransferase; Carnitine O-Palmitoyltransferase; Diabetes, Gestational; Fatty Acids, Nonesterified; Female; Gene Expression Regulation; Humans; Infant, Newborn; Insulin Resistance; Monocytes; Organic Cation Transport Proteins; Pregnancy; Pregnancy Trimester, Second; RNA, Messenger; Solute Carrier Family 22 Member 5

2009
Short-term effects of dietary fatty acids on muscle lipid composition and serum acylcarnitine profile in human subjects.
    Obesity (Silver Spring, Md.), 2011, Volume: 19, Issue:2

    Topics: Adipose Tissue; Carnitine; Dietary Fats; Dose-Response Relationship, Drug; Female; Humans; Male; Muscle, Skeletal; Oleic Acid; Oxidation-Reduction; Palmitic Acid; Triglycerides

2011
Medium chain acylcarnitines dominate the metabolite pattern in humans under moderate intensity exercise and support lipid oxidation.
    PloS one, 2010, Jul-12, Volume: 5, Issue:7

    Topics: Adult; Animals; Carnitine; Cells, Cultured; Chromatography, High Pressure Liquid; Exercise; Humans; In Vitro Techniques; Lipid Metabolism; Magnetic Resonance Spectroscopy; Male; Mass Spectrometry; Mice; Muscle Fibers, Skeletal; Muscle, Skeletal; Oxidation-Reduction; Reverse Transcriptase Polymerase Chain Reaction

2010
L-carnitine supplementation and adipokines in patients with end-stage renal disease on regular hemodialysis.
    Experimental and clinical endocrinology & diabetes : official journal, German Society of Endocrinology [and] German Diabetes Association, 2010, Volume: 118, Issue:10

    Topics: Adipokines; Aged; Aged, 80 and over; Arteriosclerosis; Carnitine; Female; Humans; Infusions, Intravenous; Insulin; Kidney Failure, Chronic; Male; Middle Aged; Renal Dialysis; Time Factors

2010
Effect of caloric restriction with and without exercise on metabolic intermediates in nonobese men and women.
    The Journal of clinical endocrinology and metabolism, 2011, Volume: 96, Issue:2

    Topics: Adiposity; Adult; Aging; Amino Acids; Body Composition; Caloric Restriction; Carnitine; Cross-Sectional Studies; Data Interpretation, Statistical; Exercise; Fatty Acids; Female; Humans; Insulin Resistance; Male; Metabolism; Middle Aged; Overweight; Principal Component Analysis; Sex Characteristics; Spectrum Analysis; Weight Loss

2011
Differential metabolic impact of gastric bypass surgery versus dietary intervention in obese diabetic subjects despite identical weight loss.
    Science translational medicine, 2011, Apr-27, Volume: 3, Issue:80

    Topics: Adult; Amino Acids, Branched-Chain; Carnitine; Cohort Studies; Diabetes Mellitus, Type 2; Female; Gastric Bypass; Humans; Male; Metabolic Networks and Pathways; Middle Aged; Obesity; Principal Component Analysis; Regression Analysis; Weight Loss

2011
Impaired fasting tolerance among Alaska native children with a common carnitine palmitoyltransferase 1A sequence variant.
    Molecular genetics and metabolism, 2011, Volume: 104, Issue:3

    Topics: Alaska; American Indian or Alaska Native; Body Mass Index; Carnitine; Carnitine O-Palmitoyltransferase; Child, Preschool; Fatty Acids, Nonesterified; Gas Chromatography-Mass Spectrometry; Homozygote; Humans; Hypoglycemia; Ketones; Lipid Metabolism, Inborn Errors; Polymorphism, Single Nucleotide; Tandem Mass Spectrometry

2011
Postprandial changes in plasma acylcarnitine concentrations as markers of fatty acid flux in overweight and obesity.
    Metabolism: clinical and experimental, 2012, Volume: 61, Issue:2

    Topics: Adult; Aged; Biological Transport; Biomarkers; Body Composition; Carnitine; Fatty Acids; Female; Humans; Lipid Metabolism; Male; Middle Aged; Obesity; Osmolar Concentration; Overweight; Postprandial Period; Young Adult

2012
The metabolic effects of two different lipid emulsions used in parenterally fed premature infants--a randomized comparative study.
    Early human development, 2012, Volume: 88, Issue:7

    Topics: Body Weight; Carnitine; Emulsions; Fat Emulsions, Intravenous; Female; Gestational Age; Humans; Infant, Newborn; Infant, Premature; Lipids; Male; Metabolism; Parenteral Nutrition; Phospholipids; Plant Oils; Soybean Oil

2012
Caloric restriction alters the metabolic response to a mixed-meal: results from a randomized, controlled trial.
    PloS one, 2012, Volume: 7, Issue:4

    Topics: Adult; Amino Acids; Caloric Restriction; Carnitine; Energy Intake; Exercise; Fasting; Fatty Acids, Nonesterified; Female; Glucose Tolerance Test; Humans; Insulin Resistance; Male; Middle Aged; Overweight; Postprandial Period; Principal Component Analysis

2012
Free carnitine and acylcarnitines in obese patients with polycystic ovary syndrome and effects of pioglitazone treatment.
    Fertility and sterility, 2012, Volume: 98, Issue:6

    Topics: Adult; Carnitine; Female; Humans; Hypoglycemic Agents; Obesity; Pioglitazone; Polycystic Ovary Syndrome; Single-Blind Method; Thiazolidinediones; Treatment Outcome

2012
Metabolomic profiling of urine: response to a randomised, controlled feeding study of select fruits and vegetables, and application to an observational study.
    The British journal of nutrition, 2013, Volume: 110, Issue:10

    Topics: Adult; Biomarkers; Brassicaceae; Carnitine; Citrus; Cross-Sectional Studies; Diet; Diet Records; Fatty Acids; Feeding Behavior; Female; Fruit; Glycine max; Humans; Ions; Male; Metabolome; Metabolomics; Niacin; Nutrition Assessment; Phytochemicals; Proline; Riboflavin; Surveys and Questionnaires; Vegetables; Young Adult

2013
Metabolomic analysis reveals extended metabolic consequences of marginal vitamin B-6 deficiency in healthy human subjects.
    PloS one, 2013, Volume: 8, Issue:6

    Topics: Acetates; Adult; Amino Acids; Carnitine; Cystathionine; Female; Glutamic Acid; Glutamine; Humans; Ketoglutaric Acids; Male; Metabolomics; Proline; Pyridoxal Phosphate; Vitamin B 6; Vitamin B 6 Deficiency; Young Adult

2013
Differences in circulating carnitine status of preterm infants fed fortified human milk or preterm infant formula.
    Journal of pediatric gastroenterology and nutrition, 2013, Volume: 57, Issue:5

    Topics: Carnitine; Child Development; Esterification; Female; Food, Fortified; Germany; Humans; Infant Formula; Infant Nutritional Physiological Phenomena; Infant, Low Birth Weight; Infant, Newborn; Infant, Premature; Infant, Very Low Birth Weight; Longitudinal Studies; Male; Milk, Human; Nutritional Status

2013
The metabolic signature associated with the Western dietary pattern: a cross-sectional study.
    Nutrition journal, 2013, Dec-11, Volume: 12

    Topics: Adult; Amino Acids, Branched-Chain; Cardiovascular Diseases; Carnitine; Cohort Studies; Cross-Sectional Studies; Diet; Energy Metabolism; Fatty Acids, Volatile; Feeding Behavior; Female; Health Promotion; Humans; Male; Metabolomics; Nutrition Policy; Patient Compliance; Principal Component Analysis; Quebec; Risk Factors; Up-Regulation; Urban Health

2013
Dietary intake of palmitate and oleate has broad impact on systemic and tissue lipid profiles in humans.
    The American journal of clinical nutrition, 2014, Volume: 99, Issue:3

    Topics: Adolescent; Adult; Cardiovascular Diseases; Carnitine; Cohort Studies; Cross-Over Studies; Dietary Fats; Female; Humans; Intracellular Signaling Peptides and Proteins; Lipid Metabolism; Lipids; Male; Membrane Proteins; Metabolomics; Muscle, Skeletal; Oleic Acid; Palmitic Acid; Risk Factors; Sex Characteristics; Up-Regulation; Vermont; Young Adult

2014
Dietary protein intake affects amino acid and acylcarnitine metabolism in infants aged 6 months.
    The Journal of clinical endocrinology and metabolism, 2015, Volume: 100, Issue:1

    Topics: Amino Acids; Body Weight; Breast Feeding; Carnitine; Dietary Proteins; Double-Blind Method; Female; Humans; Infant; Infant Food; Male; Obesity

2015
Effects of levocarnitine on brachial-ankle pulse wave velocity in hemodialysis patients: a randomized controlled trial.
    Nutrients, 2014, Dec-22, Volume: 6, Issue:12

    Topics: Administration, Oral; Aged; Ankle Brachial Index; Atherosclerosis; Biomarkers; Blood Flow Velocity; Carnitine; Dietary Supplements; Female; Humans; Kidney Failure, Chronic; Male; Middle Aged; Prospective Studies; Pulsatile Flow; Renal Dialysis

2014
Metabolic responses of healthy or prediabetic adults to bovine whey protein and sodium caseinate do not differ.
    The Journal of nutrition, 2015, Volume: 145, Issue:3

    Topics: Adult; Amino Acids; Blood Glucose; C-Peptide; Carnitine; Caseins; Dietary Carbohydrates; Female; Gastric Emptying; Gastric Inhibitory Polypeptide; Glucagon; Glucagon-Like Peptide 1; Healthy Volunteers; Humans; Insulin; Male; Milk Proteins; Postprandial Period; Prediabetic State; Single-Blind Method; Whey Proteins

2015
Increased palmitate intake: higher acylcarnitine concentrations without impaired progression of β-oxidation.
    Journal of lipid research, 2015, Volume: 56, Issue:9

    Topics: Adolescent; Adult; Body Composition; Carnitine; Cytokines; Diet; Dietary Fats; Fatty Acids; Female; Humans; Immunity, Innate; Lipid Peroxidation; Male; Oleic Acid; Palmitates

2015
Type 2 diabetes is associated with postprandial amino acid measures.
    Archives of biochemistry and biophysics, 2016, Jan-01, Volume: 589

    Topics: Adult; Amino Acids; Biomarkers; Carnitine; Diabetes Mellitus, Type 2; Female; Humans; Insulin Resistance; Male; Postprandial Period; Sleep

2016
Physiological changes due to mild cooling in healthy lean males of white Caucasian and South Asian descent: A metabolomics study.
    Archives of biochemistry and biophysics, 2016, Jan-01, Volume: 589

    Topics: Adolescent; Adult; Asian People; Body Weight; Carnitine; Cold Temperature; Fatty Acids, Nonesterified; Glycerophospholipids; Humans; Male; Metabolomics; Sphingomyelins; Thermogenesis; White People; Young Adult

2016
A single night of sleep curtailment increases plasma acylcarnitines: Novel insights in the relationship between sleep and insulin resistance.
    Archives of biochemistry and biophysics, 2016, Jan-01, Volume: 589

    Topics: Adult; Carnitine; Fasting; Female; Humans; Insulin Resistance; Male; Metabolomics; Sleep

2016
Krill oil reduces plasma triacylglycerol level and improves related lipoprotein particle concentration, fatty acid composition and redox status in healthy young adults - a pilot study.
    Lipids in health and disease, 2015, Dec-15, Volume: 14

    Topics: Adolescent; Adult; Animals; Atherosclerosis; Betaine; Carnitine; Choline; Chylomicrons; Cytokines; Dietary Fats, Unsaturated; Docosahexaenoic Acids; Eicosapentaenoic Acid; Erythrocytes; Euphausiacea; Fatty Acids, Unsaturated; Female; Humans; Lipoproteins, VLDL; Male; Methylamines; Particle Size; Pilot Projects; Triglycerides

2015
Prognostic Implications of Long-Chain Acylcarnitines in Heart Failure and Reversibility With Mechanical Circulatory Support.
    Journal of the American College of Cardiology, 2016, Jan-26, Volume: 67, Issue:3

    Topics: Aged; Carnitine; Disease Progression; Energy Metabolism; Exercise Therapy; Female; Heart Failure, Systolic; Heart-Assist Devices; Hospital Mortality; Humans; Male; Mass Spectrometry; Middle Aged; North Carolina; Outcome Assessment, Health Care; Prognosis; Reproducibility of Results; Severity of Illness Index; Statistics as Topic

2016
Mortality prediction in patients with severe septic shock: a pilot study using a target metabolomics approach.
    Scientific reports, 2016, Feb-05, Volume: 6

    Topics: Aged; Aged, 80 and over; Amino Acids; Carnitine; Cohort Studies; Female; Glycerophospholipids; Gram-Negative Bacteria; Gram-Positive Bacteria; Humans; Intensive Care Units; Kynurenine; Male; Metabolome; Metabolomics; Middle Aged; Pilot Projects; Shock, Septic; Sphingolipids; Survival Analysis

2016
Plasma acylcarnitines and risk of cardiovascular disease: effect of Mediterranean diet interventions.
    The American journal of clinical nutrition, 2016, Volume: 103, Issue:6

    Topics: Aged; Body Mass Index; Cardiovascular Diseases; Carnitine; Cohort Studies; Diet, Mediterranean; Female; Humans; Male; Middle Aged; Prospective Studies; Risk Factors; Stroke

2016
Assessment of plasma acylcarnitines before and after weight loss in obese subjects.
    Archives of biochemistry and biophysics, 2016, Sep-15, Volume: 606

    Topics: Adult; Anthropometry; Body Composition; Carnitine; Diabetes Mellitus, Type 2; Fatty Acids; Fatty Acids, Nonesterified; Female; Glucose; Humans; Insulin Resistance; Lipolysis; Male; Middle Aged; Obesity; Oxygen; Respiration; Weight Loss; Young Adult

2016
Screening of carnitine and biotin deficiencies on tandem mass spectrometry.
    Pediatrics international : official journal of the Japan Pediatric Society, 2017, Volume: 59, Issue:4

    Topics: Adolescent; Biomarkers; Biotin; Carnitine; Child; Child, Preschool; Dried Blood Spot Testing; Female; Humans; Infant; Male; Mass Screening; Nutritional Status; Prospective Studies; Tandem Mass Spectrometry

2017
Reduction in cardiometabolic risk factors by a multifunctional diet is mediated via several branches of metabolism as evidenced by nontargeted metabolite profiling approach.
    Molecular nutrition & food research, 2017, Volume: 61, Issue:2

    Topics: Aged; Cardiovascular Diseases; Carnitine; Cholesterol, LDL; Diet; Fasting; Fatty Acids; Female; Humans; Lipids; Male; Metabolomics; Middle Aged; Overweight; Risk Factors; Triglycerides

2017
Effects of weight loss using supplementation with Lactobacillus strains on body fat and medium-chain acylcarnitines in overweight individuals.
    Food & function, 2017, Jan-25, Volume: 8, Issue:1

    Topics: Adipose Tissue; Carnitine; Dietary Supplements; Double-Blind Method; Humans; Lactobacillus; Lactobacillus plantarum; Overweight; Probiotics; Triglycerides; Weight Loss

2017
A discovery-driven approach to elucidate urinary metabolome changes after a regular and moderate consumption of beer and nonalcoholic beer in subjects at high cardiovascular risk.
    Molecular nutrition & food research, 2017, Volume: 61, Issue:10

    Topics: 3-Hydroxyacyl CoA Dehydrogenases; Acetyl-CoA C-Acyltransferase; Adipates; Aged; Alcohol Drinking; Beer; Beverages; Biomarkers; Carbon-Carbon Double Bond Isomerases; Cardiovascular Diseases; Carnitine; Creatinine; Cross-Over Studies; Diet; Enoyl-CoA Hydratase; Exercise; Humans; Male; Metabolome; Metabolomics; Middle Aged; Patient Compliance; Pentanoic Acids; Racemases and Epimerases; Risk Factors; Xanthones

2017
Treatment of Swedish Patients with Graves' Hyperthyroidism Is Associated with Changes in Acylcarnitine Levels.
    Thyroid : official journal of the American Thyroid Association, 2017, Volume: 27, Issue:9

    Topics: Adult; Antithyroid Agents; Carnitine; Combined Modality Therapy; Fatty Acids; Fatty Acids, Volatile; Female; Follow-Up Studies; Graves Disease; Hormone Replacement Therapy; Humans; Male; Metabolomics; Methimazole; Middle Aged; Molecular Weight; Principal Component Analysis; Sweden; Thyroid Gland; Thyroxine

2017
Military training elicits marked increases in plasma metabolomic signatures of energy metabolism, lipolysis, fatty acid oxidation, and ketogenesis.
    Physiological reports, 2017, Volume: 5, Issue:17

    Topics: Carnitine; Diglycerides; Energy Metabolism; Fatty Acids; Humans; Lipolysis; Male; Metabolome; Military Personnel; Physical Conditioning, Human; Skiing; Young Adult

2017
Plasma Palmitoyl-Carnitine (AC16:0) Is a Marker of Increased Postprandial Nonesterified Incomplete Fatty Acid Oxidation Rate in Adults With Type 2 Diabetes.
    Canadian journal of diabetes, 2018, Volume: 42, Issue:4

    Topics: Adult; Biomarkers; Carnitine; Diabetes Mellitus, Type 2; Fasting; Fatty Acids, Nonesterified; Female; Humans; Insulin Resistance; Lipid Metabolism; Male; Middle Aged; Oxidation-Reduction; Palmitoylcarnitine; Postprandial Period; Young Adult

2018
Basal insulin peglispro increases lipid oxidation, metabolic flexibility, thermogenesis and ketone bodies compared to insulin glargine in subjects with type 1 diabetes mellitus.
    Diabetes, obesity & metabolism, 2018, Volume: 20, Issue:5

    Topics: Adult; Basal Metabolism; Biomarkers; Breakfast; Carnitine; Cross-Over Studies; Diabetes Mellitus, Type 1; Drug Administration Schedule; Drug Monitoring; Female; Follow-Up Studies; Glycolysis; Humans; Hypoglycemic Agents; Insulin Glargine; Insulin Lispro; Ketone Bodies; Lipolysis; Male; Middle Aged; Oxidation-Reduction; Polyethylene Glycols; Thermogenesis; Young Adult

2018
Plasma fatty acyl-carnitines during 8 weeks of overfeeding: relation to diet energy expenditure and body composition: the PROOF study.
    Metabolism: clinical and experimental, 2018, Volume: 83

    Topics: Adolescent; Adult; Body Composition; Carnitine; Diet; Energy Intake; Energy Metabolism; Fatty Acids; Female; Humans; Hyperphagia; Male; Young Adult

2018
Family history and obesity in youth, their effect on acylcarnitine/aminoacids metabolomics and non-alcoholic fatty liver disease (NAFLD). Structural equation modeling approach.
    PloS one, 2018, Volume: 13, Issue:2

    Topics: Adolescent; Adult; Amino Acids; Carnitine; Cross-Sectional Studies; Diabetes Mellitus, Type 2; Female; Humans; Male; Medical History Taking; Metabolomics; Middle Aged; Models, Biological; Non-alcoholic Fatty Liver Disease; Obesity

2018
Metabolic perturbations associated with the consumption of a ketogenic medium-chain TAG diet in dogs with idiopathic epilepsy.
    The British journal of nutrition, 2018, Volume: 120, Issue:5

    Topics: Animals; Anticonvulsants; Carnitine; Chromatography, Liquid; Cross-Over Studies; Diet; Diet, Ketogenic; Dog Diseases; Dogs; Epilepsy; Fasting; Fatty Acids; Female; Lipid Metabolism; Lipids; Male; Mass Spectrometry; Metabolome; Phosphatidylcholines; Placebos; Triglycerides

2018
GS-0976 Reduces Hepatic Steatosis and Fibrosis Markers in Patients With Nonalcoholic Fatty Liver Disease.
    Gastroenterology, 2018, Volume: 155, Issue:5

    Topics: Acetyl-CoA Carboxylase; Biomarkers; Carnitine; Double-Blind Method; Elasticity Imaging Techniques; Fatty Liver; Female; Humans; Isobutyrates; Liver Cirrhosis; Magnetic Resonance Imaging; Male; Middle Aged; Non-alcoholic Fatty Liver Disease; Oxazoles; Pyrimidines

2018
The Association of Acylcarnitines and Amino Acids With Age in Dutch and South-Asian Surinamese Living in Amsterdam.
    The Journal of clinical endocrinology and metabolism, 2018, 10-01, Volume: 103, Issue:10

    Topics: Adolescent; Adult; Age Factors; Aged; Amino Acids; Asian People; Biomarkers; Carnitine; Cohort Studies; Cross-Sectional Studies; Ethnicity; Female; Follow-Up Studies; Humans; Male; Metabolic Diseases; Middle Aged; Netherlands; Prevalence; Prognosis; Suriname; White People; Young Adult

2018
Plasma metabolomic profiling of a ketamine and placebo crossover trial of major depressive disorder and healthy control subjects.
    Psychopharmacology, 2018, Volume: 235, Issue:10

    Topics: Adult; Amino Acids, Essential; Antidepressive Agents; Biogenic Amines; Carnitine; Cross-Over Studies; Depressive Disorder, Major; Depressive Disorder, Treatment-Resistant; Double-Blind Method; Female; Glycerophospholipids; Humans; Ketamine; Lipids; Male; Metabolomics; Middle Aged; Sphingolipids; Young Adult

2018
Changes in Plasma Acylcarnitine and Lysophosphatidylcholine Levels Following a High-Fructose Diet: A Targeted Metabolomics Study in Healthy Women.
    Nutrients, 2018, Sep-06, Volume: 10, Issue:9

    Topics: Adult; Biomarkers; Carnitine; Cross-Over Studies; Diet, Carbohydrate-Restricted; Dietary Sugars; Female; Fruit; Germany; High Fructose Corn Syrup; Humans; Lipid Peroxidation; Lysophosphatidylcholines; Metabolomics; Mitochondria; Obesity; Oxidation-Reduction; Time Factors; Vegetables; Young Adult

2018
Metabolomics identifies increases in the acylcarnitine profiles in the plasma of overweight subjects in response to mild weight loss: a randomized, controlled design study.
    Lipids in health and disease, 2018, Oct-15, Volume: 17, Issue:1

    Topics: Adult; Caloric Restriction; Carnitine; Fatty Acids, Nonesterified; Female; Humans; Intra-Abdominal Fat; Male; Metabolomics; Middle Aged; Overweight; Weight Loss

2018
An Integrated Understanding of the Molecular Mechanisms of How Adipose Tissue Metabolism Affects Long-term Body Weight Maintenance.
    Diabetes, 2019, Volume: 68, Issue:1

    Topics: Adipose Tissue; Adolescent; Body Weight; Body Weight Maintenance; Carnitine; Child; Female; Humans; Male; Weight Loss

2019
Nutritional ketosis improves exercise metabolism in patients with very long-chain acyl-CoA dehydrogenase deficiency.
    Journal of inherited metabolic disease, 2020, Volume: 43, Issue:4

    Topics: Adolescent; Adult; Beverages; Blood Glucose; Carnitine; Congenital Bone Marrow Failure Syndromes; Cross-Over Studies; Diet, Ketogenic; Endurance Training; Esters; Exercise Test; Female; Humans; Ketones; Ketosis; Lipid Metabolism, Inborn Errors; Magnetic Resonance Spectroscopy; Male; Middle Aged; Mitochondrial Diseases; Muscle, Skeletal; Muscular Diseases; Netherlands; Pulmonary Gas Exchange; Young Adult

2020
Alterations of the acylcarnitine profiles in blood serum and in muscle from periparturient cows with normal or elevated body condition.
    Journal of dairy science, 2020, Volume: 103, Issue:5

    Topics: Animals; Carnitine; Carnitine O-Palmitoyltransferase; Cattle; Diet; Fatty Acids; Female; Lactation; Muscles; Parturition; Pregnancy

2020
Altered Plasma Acylcarnitines and Amino Acids Profile in Spinocerebellar Ataxia Type 7.
    Biomolecules, 2020, 03-03, Volume: 10, Issue:3

    Topics: Adult; Amino Acids; Biomarkers; Carnitine; Female; Humans; Male; Middle Aged; Spinocerebellar Ataxias

2020
Metabolome and microbiome alterations related to short-term feeding of a micronutrient-fortified, high-quality legume protein-based food product to stunted school age children: A randomized controlled pilot trial.
    Clinical nutrition (Edinburgh, Scotland), 2020, Volume: 39, Issue:11

    Topics: Amino Acids; Anthropometry; Carnitine; Child; Diet, High-Protein; Eating; Fabaceae; Feces; Female; Food, Fortified; Gastrointestinal Microbiome; Growth Disorders; Humans; Male; Metabolome; Micronutrients; Pilot Projects; Treatment Outcome

2020
A metabolomic study of red and processed meat intake and acylcarnitine concentrations in human urine and blood.
    The American journal of clinical nutrition, 2020, 08-01, Volume: 112, Issue:2

    Topics: Adult; Animals; Carnitine; Cross-Sectional Studies; Female; Humans; Male; Meat Products; Metabolomics; Prospective Studies; Swine

2020
L-carnitine infusion does not alleviate lipid-induced insulin resistance and metabolic inflexibility.
    PloS one, 2020, Volume: 15, Issue:9

    Topics: Adult; Carnitine; Cross-Over Studies; Emulsions; Fatty Acids, Nonesterified; Humans; Infusion Pumps; Insulin; Insulin Resistance; Lipids; Male; Muscle, Skeletal; Phospholipids; Soybean Oil; Young Adult

2020
Different Blood Metabolomics Profiles in Infants Consuming a Meat- or Dairy-Based Complementary Diet.
    Nutrients, 2021, Jan-27, Volume: 13, Issue:2

    Topics: Amino Acids, Branched-Chain; Amino Acids, Essential; Carnitine; Dairy Products; Diet; Follow-Up Studies; Humans; Infant; Infant Nutritional Physiological Phenomena; Isoleucine; Leucine; Meat; Metabolomics; Phenylalanine; Valine

2021
Plasma acylcarnitines and risk of lower-extremity functional impairment in older adults: a nested case-control study.
    Scientific reports, 2021, 02-08, Volume: 11, Issue:1

    Topics: Aged; Body Mass Index; Carnitine; Energy Intake; Female; Humans; Lower Extremity; Male; Middle Aged

2021
Metabolite profiles associated with disease progression in influenza infection.
    PloS one, 2021, Volume: 16, Issue:4

    Topics: Adult; Carnitine; Case-Control Studies; Disease Progression; Female; Glycerides; Humans; Influenza A Virus, H1N1 Subtype; Influenza, Human; Male; Metabolome; Middle Aged; Sphingolipids

2021
Urinary Medium-Chained Acyl-Carnitines Sign High Caloric Intake whereas Short-Chained Acyl-Carnitines Sign High -Protein Diet within a High-Fat, Hypercaloric Diet in a Randomized Crossover Design Dietary Trial.
    Nutrients, 2021, Apr-03, Volume: 13, Issue:4

    Topics: Adult; Biomarkers; Carnitine; Cross-Over Studies; Diet, High-Fat; Diet, High-Protein; Diet, Western; Dietary Fats; Dietary Proteins; Energy Intake; Female; Glucose; Healthy Volunteers; Humans; Lipid Metabolism; Liver; Male; Metabolic Syndrome; Postprandial Period; Renal Elimination; Young Adult

2021
Effects of gut microbiota and fatty acid metabolism on dyslipidemia following weight-loss diets in women: Results from a randomized controlled trial.
    Clinical nutrition (Edinburgh, Scotland), 2021, Volume: 40, Issue:11

    Topics: Adult; Caloric Restriction; Carnitine; Diet, Carbohydrate-Restricted; Diet, Reducing; Dyslipidemias; Erythrocytes; Fatty Acids; Feces; Female; Gastrointestinal Microbiome; Humans; Lipid Metabolism; Middle Aged; Obesity; Overweight; Treatment Outcome; Weight Loss

2021
Plasma acylcarnitines and risk of incident heart failure and atrial fibrillation: the Prevención con dieta mediterránea study.
    Revista espanola de cardiologia (English ed.), 2022, Volume: 75, Issue:8

    Topics: Atrial Fibrillation; Cardiovascular Diseases; Carnitine; Diabetes Mellitus, Type 2; Diet, Mediterranean; Heart Failure; Humans; Mediterranea; Nuts; Obesity; Olive Oil; Risk Factors

2022
Gut microbiota and acylcarnitine metabolites connect the beneficial association between equol and adiposity in adults: a prospective cohort study.
    The American journal of clinical nutrition, 2022, 12-19, Volume: 116, Issue:6

    Topics: Adiposity; Adult; Carnitine; Equol; Female; Gastrointestinal Microbiome; Humans; Isoflavones; Male; Middle Aged; Obesity; Prospective Studies

2022
S-adenosyl methionine (SAMe) versus escitalopram and placebo in major depression RCT: efficacy and effects of histamine and carnitine as moderators of response.
    Journal of affective disorders, 2014, Volume: 164

    Topics: Adult; Antidepressive Agents; Biomarkers; Carnitine; Citalopram; Depressive Disorder, Major; Double-Blind Method; Female; Histamine; Humans; Male; Middle Aged; S-Adenosylmethionine; Selective Serotonin Reuptake Inhibitors; Treatment Outcome

2014

Other Studies

977 other study(ies) available for carnitine and s-adenosylmethionine

ArticleYear
Studies on the oxidation of phytanic acid and pristanic acid in human fibroblasts by acylcarnitine analysis.
    Journal of inherited metabolic disease, 1998, Volume: 21, Issue:7

    Topics: Carnitine; Cells, Cultured; Fatty Acids; Fibroblasts; Humans; Oxidation-Reduction; Peroxisomal Disorders; Phytanic Acid

1998
Low levels of serum acylcarnitine in chronic fatigue syndrome and chronic hepatitis type C, but not seen in other diseases.
    International journal of molecular medicine, 1998, Volume: 2, Issue:1

    Topics: Acute Disease; Animals; Carnitine; Chemical and Drug Induced Liver Injury; Diabetes Mellitus; Fatigue Syndrome, Chronic; Female; Galactosamine; Hepatitis C, Chronic; Humans; Japan; Male; Mice; Mice, Inbred C3H; Neoplasms; Sweden

1998
Short-chain acyl-CoA dehydrogenase deficiency: a cause of ophthalmoplegia and multicore myopathy.
    Neurology, 1999, Jan-15, Volume: 52, Issue:2

    Topics: Acyl-CoA Dehydrogenase; Acyl-CoA Dehydrogenases; Adolescent; Carnitine; Cells, Cultured; Diagnosis, Differential; Female; Fibroblasts; Humans; Mitochondrial Myopathies; Ophthalmoplegia, Chronic Progressive External; Phenotype

1999
Cardiomegaly in the juvenile visceral steatosis (JVS) mouse is reduced with acute elevation of heart short-chain acyl-carnitine level after L-carnitine injection.
    FEBS letters, 1999, Jan-29, Volume: 443, Issue:3

    Topics: Aging; Animals; Body Weight; Cardiomegaly; Carnitine; Disease Models, Animal; Half-Life; Injections, Intraperitoneal; Lipid Metabolism; Mice; Mice, Inbred Strains; Myocardium; Organ Size; Time Factors

1999
Carnitine homeostasis in patients with rheumatoid arthritis.
    Clinica chimica acta; international journal of clinical chemistry, 1999, Volume: 279, Issue:1-2

    Topics: Adult; Aged; Arthritis, Rheumatoid; Biomechanical Phenomena; Carnitine; Female; Homeostasis; Humans; Kidney; Male; Middle Aged; Muscle, Skeletal

1999
Identification of new medium-chain acylcarnitines present in urine of a patient with medium-chain acyl-CoA dehydrogenase deficiency.
    Journal of inherited metabolic disease, 1999, Volume: 22, Issue:1

    Topics: Acyl-CoA Dehydrogenase; Acyl-CoA Dehydrogenases; Carnitine; Child, Preschool; Female; Glucuronates; Glycine; Humans

1999
Normal acylcarnitines in maternal urine during a pregnancy affected by glutaric aciduria type II.
    Journal of inherited metabolic disease, 1999, Volume: 22, Issue:1

    Topics: Acidosis, Renal Tubular; Carnitine; Female; Glutarates; Humans; Infant, Newborn; Male; Pregnancy

1999
Evaluation of carnitine nutritional status in full-term newborn infants.
    Early human development, 1998, Volume: 53 Suppl

    Topics: Carnitine; Dietary Supplements; Humans; Infant Food; Infant, Newborn; Nutritional Status; Reference Values

1998
Quantitative acylcarnitine profiling in fibroblasts using [U-13C] palmitic acid: an improved tool for the diagnosis of fatty acid oxidation defects.
    Clinica chimica acta; international journal of clinical chemistry, 1999, Volume: 281, Issue:1-2

    Topics: Carbon Isotopes; Carnitine; Cells, Cultured; Fatty Acids; Fibroblasts; Gas Chromatography-Mass Spectrometry; Humans; Lipid Metabolism, Inborn Errors; Oxidation-Reduction; Palmitic Acid; Spectrometry, Mass, Fast Atom Bombardment

1999
Influence of acetate and bicarbonate dialysate on blood short- and long-chain acyl carnitine in adult pyelonephritis patients.
    Annals of clinical biochemistry, 1999, Volume: 36 ( Pt 1)

    Topics: Acetates; Adult; Bicarbonates; Carnitine; Chronic Disease; Erythrocytes; Female; Humans; Male; Pyelonephritis; Renal Dialysis

1999
Carnitine-acylcarnitine translocase deficiency is a treatable disease.
    Journal of inherited metabolic disease, 1999, Volume: 22, Issue:3

    Topics: Acetylcarnitine; Carnitine; Carnitine Acyltransferases; Female; Humans; Infant, Newborn; Lipid Metabolism, Inborn Errors; Palmitoylcarnitine; Treatment Outcome

1999
Quantitative plasma acylcarnitine analysis using electrospray tandem mass spectrometry for the diagnosis of organic acidaemias and fatty acid oxidation defects.
    Journal of inherited metabolic disease, 1999, Volume: 22, Issue:3

    Topics: Carnitine; Child; Child, Preschool; Fatty Acids; Humans; Infant; Lipid Metabolism, Inborn Errors; Mass Spectrometry; Oxidation-Reduction

1999
Diagnosis of mitochondrial trifunctional protein deficiency in a blood spot from the newborn screening card by tandem mass spectrometry and DNA analysis.
    Pediatric research, 1999, Volume: 46, Issue:1

    Topics: 3-Hydroxyacyl CoA Dehydrogenases; Base Sequence; Cardiomyopathies; Carnitine; DNA; Female; Genetic Testing; Heterozygote; Humans; Infant, Newborn; Long-Chain-3-Hydroxyacyl-CoA Dehydrogenase; Male; Mass Spectrometry; Mitochondrial Trifunctional Protein; Multienzyme Complexes; Mutation, Missense; Neonatal Screening; Nuclear Family; Reye Syndrome; Sequence Deletion

1999
Very long chain acyl coenzyme A dehydrogenase deficiency in a 5-month-old Korean boy: identification of a novel mutation.
    The Journal of pediatrics, 1999, Volume: 135, Issue:2 Pt 1

    Topics: Acyl-CoA Dehydrogenase, Long-Chain; Arginine; Cardiomyopathies; Carnitine; Codon, Terminator; Fatal Outcome; Fatty Acid Desaturases; Genetic Heterogeneity; Humans; Infant; Korea; Lipid Metabolism, Inborn Errors; Male; Mutation

1999
Noninvasive evaluation of adult onset myopathy from carnitine palmitoyl transferase II deficiency using proton magnetic resonance spectroscopy.
    The Journal of rheumatology, 1999, Volume: 26, Issue:8

    Topics: Adult; Age of Onset; Biomarkers; Carnitine; Carnitine O-Palmitoyltransferase; Fatigue; Female; Humans; Magnetic Resonance Spectroscopy; Muscles; Muscular Diseases; Myoglobinuria

1999
Effects of pentanoate, 4-pentenoate, 3-hydroxybutyrate and insulin on the tissue-levels of long-chain acyl CoA and acylcarnitine in the oxygenated and hypoxic rat atria.
    Archives of physiology and biochemistry, 1999, Volume: 107, Issue:1

    Topics: 3-Hydroxybutyric Acid; Acyl Coenzyme A; Aerobiosis; Animals; Carnitine; Cell Hypoxia; Fatty Acids, Monounsaturated; Female; Heart Atria; Insulin; Male; Oxidative Stress; Oxygen; Pentanoic Acids; Rats; Rats, Wistar

1999
Carnitine transport and its inhibition by sulfonylureas in human kidney proximal tubular epithelial cells.
    Biochemical pharmacology, 1999, Oct-15, Volume: 58, Issue:8

    Topics: Administration, Oral; Animals; Biological Transport; Carnitine; Cell Line; Cell Membrane; Epithelial Cells; Humans; Hypoglycemic Agents; Kidney Tubules, Proximal; Microvilli; Rats; Sulfonylurea Compounds

1999
Serum carnitine concentrations in patients with idiopathic hypertrophic cardiomyopathy: relationship with impaired myocardial fatty acid metabolism.
    Clinical science (London, England : 1979), 1999, Volume: 97, Issue:4

    Topics: Adolescent; Adult; Aged; Biomarkers; Cardiomyopathy, Hypertrophic; Carnitine; Fatty Acids; Fatty Acids, Nonesterified; Female; Humans; Iodobenzenes; Male; Middle Aged; Myocardium; Radionuclide Imaging; Ultrasonography

1999
Effect of sports activity on carnitine metabolism. Measurement of free carnitine, gamma-butyrobetaine and acylcarnitines by tandem mass spectrometry.
    Journal of chromatography. B, Biomedical sciences and applications, 1999, Aug-06, Volume: 731, Issue:1

    Topics: Betaine; Carnitine; Exercise; Humans; Mass Spectrometry

1999
Acylcarnitine analogues as topical, microbicidal spermicides.
    Bioorganic & medicinal chemistry letters, 1999, Sep-06, Volume: 9, Issue:17

    Topics: Anti-Infective Agents; Candida albicans; Carnitine; HIV-1; Microbial Sensitivity Tests; Spermatocidal Agents

1999
Dynamic changes of plasma acylcarnitine levels induced by fasting and sunflower oil challenge test in children.
    Pediatric research, 1999, Volume: 46, Issue:4

    Topics: 3-Hydroxybutyric Acid; Acetoacetates; Blood Glucose; Carnitine; Child; Child, Preschool; Dietary Fats, Unsaturated; Fasting; Fatty Acids, Nonesterified; Humans; Infant; Ketone Bodies; Molecular Structure; Plant Oils; Sunflower Oil

1999
Identification of two novel mutations in the hypoglycemic phenotype of very long chain acyl-CoA dehydrogenase deficiency.
    Biochemical and biophysical research communications, 1999, Oct-22, Volume: 264, Issue:2

    Topics: Acyl-CoA Dehydrogenase, Long-Chain; Alleles; Carnitine; Child, Preschool; DNA, Complementary; Exons; Female; Fibroblasts; Humans; Hypoglycemia; Mitochondria; Mutation; Palmitoylcarnitine; Phenotype

1999
Direct chromatographic resolution of carnitine and O-acylcarnitine enantiomers on a teicoplanin-bonded chiral stationary phase.
    Journal of chromatography. A, 1999, Oct-01, Volume: 857, Issue:1-2

    Topics: Carnitine; Chromatography, High Pressure Liquid; Humans; Spectrophotometry, Ultraviolet; Stereoisomerism; Teicoplanin

1999
Arrhythmias and conduction defects as presenting symptoms of fatty acid oxidation disorders in children.
    Circulation, 1999, Nov-30, Volume: 100, Issue:22

    Topics: 3-Hydroxyacyl CoA Dehydrogenases; Acyl-CoA Dehydrogenase; Acyl-CoA Dehydrogenase, Long-Chain; Arrhythmias, Cardiac; Carnitine; Carnitine Acyltransferases; Carnitine O-Palmitoyltransferase; Fatty Acid Desaturases; Fatty Acids; Female; Heart Conduction System; Humans; Infant; Infant, Newborn; Long-Chain-3-Hydroxyacyl-CoA Dehydrogenase; Male; Mitochondria, Heart; Mitochondrial Myopathies; Models, Biological; Oxidation-Reduction; Sudden Infant Death; Tachycardia, Ventricular

1999
Influence of acetate and bicarbonate dialysate on acyl carnitine in pyelonephritis.
    Annals of clinical biochemistry, 1999, Volume: 36 ( Pt 6)

    Topics: Acetates; Bicarbonates; Carnitine; Humans; Pyelonephritis; Renal Dialysis

1999
Automated tandem mass spectrometry for mass newborn screening for disorders in fatty acid, organic acid, and amino acid metabolism.
    Journal of child neurology, 1999, Volume: 14 Suppl 1

    Topics: Amino Acids; Biomarkers; Carnitine; Humans; Infant, Newborn; Mass Screening; Mass Spectrometry; Metabolism, Inborn Errors; Prospective Studies

1999
Identification of the mitochondrial carnitine carrier in Saccharomyces cerevisiae.
    FEBS letters, 1999, Dec-03, Volume: 462, Issue:3

    Topics: Acetylcarnitine; Amino Acid Transport Systems; Carnitine; Carrier Proteins; Chromatography, Affinity; Kinetics; Mitochondria; Mitochondrial Proteins; Molecular Sequence Data; Recombinant Proteins; Saccharomyces cerevisiae; Saccharomyces cerevisiae Proteins; Substrate Specificity; Time Factors

1999
Newborn screening with tandem mass spectrometry: 12 months' experience in NSW Australia.
    Acta paediatrica (Oslo, Norway : 1992). Supplement, 1999, Volume: 88, Issue:432

    Topics: Amino Acid Metabolism, Inborn Errors; Carnitine; Female; Humans; Infant, Newborn; Male; Mass Spectrometry; Neonatal Screening; Pilot Projects; Program Evaluation; Sensitivity and Specificity; South Australia; Western Australia

1999
Evidence for a short-chain carnitine-acylcarnitine translocase in mitochondria specifically related to the metabolism of branched-chain amino acids.
    Molecular genetics and metabolism, 2000, Volume: 69, Issue:1

    Topics: Amino Acids, Branched-Chain; Biological Transport; Carnitine; Carnitine Acyltransferases; Cells, Cultured; Child; Deuterium; Fibroblasts; Humans; Intracellular Membranes; Isoleucine; Mass Spectrometry; Metabolism, Inborn Errors; Mitochondria; Oxidation-Reduction

2000
Determination of carnitine and acylcarnitines in biological samples by capillary electrophoresis-mass spectrometry.
    Journal of chromatography. B, Biomedical sciences and applications, 1999, Dec-10, Volume: 735, Issue:2

    Topics: Carnitine; Electrophoresis, Capillary; Humans; Mass Spectrometry; Reproducibility of Results; Sensitivity and Specificity

1999
Acylcarnitines in fibroblasts of patients with long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency and other fatty acid oxidation disorders.
    Journal of inherited metabolic disease, 2000, Volume: 23, Issue:1

    Topics: 3-Hydroxyacyl CoA Dehydrogenases; Carnitine; Cells, Cultured; Fatty Acids; Fibroblasts; Humans; Microbodies; Oxidation-Reduction

2000
Production and export of acylcarnitine esters by neonatal rat hepatocytes.
    Advances in experimental medicine and biology, 1999, Volume: 466

    Topics: Animals; Animals, Newborn; Carbon Radioisotopes; Carnitine; Cells, Cultured; Liver; Palmitic Acid; Rats

1999
Rapid diagnosis of organic acidemias and fatty-acid oxidation defects by quantitative electrospray tandem-MS acyl-carnitine analysis in plasma.
    Advances in experimental medicine and biology, 1999, Volume: 466

    Topics: Acyl-CoA Dehydrogenases; Biomarkers; Calibration; Carnitine; Carnitine O-Palmitoyltransferase; Humans; Lipid Metabolism, Inborn Errors; Oxidation-Reduction; Sensitivity and Specificity; Spectrometry, Mass, Secondary Ion

1999
MCAD deficiency. Acylcarnitines (AC) by tandem mass spectrometry (MS-MS) are useful to monitor dietary treatment.
    Advances in experimental medicine and biology, 1999, Volume: 466

    Topics: Acyl-CoA Dehydrogenase; Carnitine; Fatty Acid Desaturases; Female; Humans; Infant; Lipid Metabolism, Inborn Errors; Mass Spectrometry; Monitoring, Physiologic

1999
Determination of urinary acylcarnitines: a complementary aid for the high-risk screening of several organic acidurias using a simple and reliable GC/MS-based method.
    Clinical biochemistry, 2000, Volume: 33, Issue:2

    Topics: Carboxylic Acids; Carnitine; Gas Chromatography-Mass Spectrometry; Humans; Mass Screening; Mass Spectrometry; Metabolism, Inborn Errors; Reproducibility of Results; Spectrometry, Mass, Fast Atom Bombardment

2000
Identification of undescribed medium-chain acylcarnitines present in urine of patients with propionic and methylmalonic acidemias.
    Clinica chimica acta; international journal of clinical chemistry, 2000, Volume: 295, Issue:1-2

    Topics: Adult; Carboxy-Lyases; Carnitine; Case-Control Studies; Gas Chromatography-Mass Spectrometry; Humans; Infant; Lipid Metabolism, Inborn Errors; Male; Methylmalonic Acid; Methylmalonyl-CoA Decarboxylase; Propionates; Spectrometry, Mass, Fast Atom Bombardment

2000
Glutaric aciduria type II: observations in seven patients with neonatal- and late-onset disease.
    Journal of perinatology : official journal of the California Perinatal Association, 2000, Volume: 20, Issue:2

    Topics: Acidosis; Age of Onset; Brain; Carnitine; Child; Consanguinity; Female; Glutarates; Humans; Infant; Infant, Newborn; Magnetic Resonance Imaging; Male; Mass Spectrometry; Metabolism, Inborn Errors; Tomography, Emission-Computed

2000
Very-long-chain acyl-coenzyme A dehydrogenase (VLCAD) deficiency: monitoring of treatment by carnitine/acylcarnitine analysis in blood spots.
    Acta paediatrica (Oslo, Norway : 1992), 2000, Volume: 89, Issue:4

    Topics: Carnitine; Fatty Acid Desaturases; Humans; Infant; Lipid Metabolism, Inborn Errors; Male; Monitoring, Physiologic; Sequence Analysis, DNA

2000
2-Methylbutyryl-coenzyme A dehydrogenase deficiency: a new inborn error of L-isoleucine metabolism.
    Pediatric research, 2000, Volume: 47, Issue:6

    Topics: Amino Acid Metabolism, Inborn Errors; Base Sequence; Carnitine; DNA Primers; DNA, Complementary; Female; Humans; Infant; Isoleucine; Male; Oxidoreductases; Oxidoreductases Acting on CH-CH Group Donors; Pregnancy; Prenatal Diagnosis

2000
Metabolism of carnitine in phenylacetic acid-treated rats and in patients with phenylketonuria.
    Biochimica et biophysica acta, 2000, Jun-15, Volume: 1501, Issue:2-3

    Topics: Adult; Animals; Betaine; Carnitine; Female; Glutamic Acid; Homogentisic Acid; Humans; Ketoglutaric Acids; Liver; Male; Mass Spectrometry; Phenylacetates; Phenylketonurias; Phenylpyruvic Acids; Rats; Rats, Wistar

2000
Normal carnitine levels in patients with chronic fatigue syndrome.
    The Netherlands journal of medicine, 2000, Volume: 57, Issue:1

    Topics: Adult; Carnitine; Case-Control Studies; Fatigue; Fatigue Syndrome, Chronic; Female; Humans; Middle Aged; Netherlands; Registries; Severity of Illness Index; Single-Blind Method; Statistics, Nonparametric

2000
Carnitine-acylcarnitine translocase deficiency: metabolic consequences of an impaired mitochondrial carnitine cycle.
    Clinica chimica acta; international journal of clinical chemistry, 2000, Volume: 298, Issue:1-2

    Topics: Ammonia; Cardiac Output, Low; Carnitine; Carnitine Acyltransferases; Cells, Cultured; Coma; Consanguinity; Dicarboxylic Acids; Fatal Outcome; Female; Fibroblasts; Humans; Hypoglycemia; Infant, Newborn; Lactic Acid; Mass Spectrometry; Mitochondria; Pregnancy; Seizures; Uric Acid

2000
The biochemical metabolite screen in the Munich ENU Mouse Mutagenesis Project: determination of amino acids and acylcarnitines by tandem mass spectrometry.
    Mammalian genome : official journal of the International Mammalian Genome Society, 2000, Volume: 11, Issue:7

    Topics: Amino Acids; Animals; Biomarkers; Carnitine; Ethylnitrosourea; Mass Spectrometry; Mice; Mutagenesis; Mutagens

2000
Long-chain acylcarnitine induces Ca2+ efflux from the sarcoplasmic reticulum.
    Journal of cardiovascular pharmacology, 2000, Volume: 36, Issue:1

    Topics: Animals; Calcium; Calcium Channels; Calcium-Transporting ATPases; Carnitine; Myocardium; Palmitoylcarnitine; Rabbits; Ryanodine Receptor Calcium Release Channel; Sarcoplasmic Reticulum

2000
Evaluation of brain long-chain acylcarnitines during cerebral ischemia.
    Lipids, 2000, Volume: 35, Issue:6

    Topics: Animals; Brain; Brain Ischemia; Carnitine; Male; Rats; Spectrometry, Mass, Electrospray Ionization

2000
Elucidation of the mechanism by which (+)-acylcarnitines inhibit mitochondrial fatty acid transport.
    The Journal of biological chemistry, 2000, Nov-24, Volume: 275, Issue:47

    Topics: Animals; Biological Transport, Active; Carnitine; Carnitine Acyltransferases; Carnitine O-Palmitoyltransferase; Cells, Cultured; Fatty Acids; Male; Mitochondria, Liver; Rats; Rats, Sprague-Dawley

2000
Kinetics of carnitine palmitoyltransferase-I are altered by dietary variables and suggest a metabolic need for supplemental carnitine in young pigs.
    The Journal of nutrition, 2000, Volume: 130, Issue:10

    Topics: Animals; Carnitine; Carnitine O-Palmitoyltransferase; Diet; Dietary Supplements; Glycine max; Kinetics; Liver; Mitochondria; Mitochondria, Liver; Muscle, Skeletal; Soybean Oil; Swine; Zea mays

2000
Acylcarnitines in plasma and blood spots of patients with long-chain 3-hydroxyacyl-coenzyme A dehydrogenase defiency.
    Journal of inherited metabolic disease, 2000, Volume: 23, Issue:6

    Topics: 3-Hydroxyacyl CoA Dehydrogenases; Carnitine; Erythrocytes; False Negative Reactions; Female; Genotype; Heterozygote; Homozygote; Humans; Male; Mutation; Plasma; Sensitivity and Specificity

2000
Myocardial carnitine and carnitine palmitoyltransferase deficiencies in patients with severe heart failure.
    Biochimica et biophysica acta, 2000, Nov-15, Volume: 1502, Issue:3

    Topics: Biopsy; Carnitine; Carnitine O-Palmitoyltransferase; Female; Heart Failure; Heart Transplantation; Humans; Male; Middle Aged; Myocardium; Stroke Volume

2000
Analysis of carnitine and acylcarnitines in urine by capillary electrophoresis.
    Journal of chromatography. A, 2000, Oct-20, Volume: 895, Issue:1-2

    Topics: Carnitine; Electrophoresis, Capillary; Humans; Methylhydrazines; Reference Standards

2000
Postnatal changes in neonatal acylcarnitine profile.
    Pediatric research, 2001, Volume: 49, Issue:1

    Topics: Carnitine; Fatty Acids; Humans; Infant, Newborn; Mass Spectrometry; Reference Standards

2001
The interaction of ferrocytochrome c with long-chain fatty acids and their CoA and carnitine esters.
    Biochemistry and cell biology = Biochimie et biologie cellulaire, 2000, Volume: 78, Issue:6

    Topics: Acyl Coenzyme A; Animals; Carnitine; Cytochrome c Group; Fatty Acids; Fatty Alcohols; Horses; Ligands; Nuclear Magnetic Resonance, Biomolecular; Oleic Acid; Palmitoylcarnitine; Spectrum Analysis

2000
Carnitine palmitoyltransferase I deficiency in neonate identified by dried blood spot free carnitine and acylcarnitine profile.
    Journal of inherited metabolic disease, 2001, Volume: 24, Issue:1

    Topics: Carnitine; Carnitine O-Palmitoyltransferase; Fibroblasts; Humans; Infant, Newborn; Male; Oxidation-Reduction

2001
Carnitine transporter defect diagnosed by newborn screening with electrospray tandem mass spectrometry.
    The Journal of pediatrics, 2001, Volume: 138, Issue:4

    Topics: Birth Weight; Carnitine; Carrier Proteins; Humans; Infant, Newborn; Male; Neonatal Screening; Organic Cation Transport Proteins; Solute Carrier Family 22 Member 5; Spectrometry, Mass, Electrospray Ionization

2001
Pig liver carnitine palmitoyltransferase I, with low Km for carnitine and high sensitivity to malonyl-CoA inhibition, is a natural chimera of rat liver and muscle enzymes.
    Biochemistry, 2001, Feb-20, Volume: 40, Issue:7

    Topics: Amino Acid Sequence; Animals; Base Sequence; Carnitine; Carnitine O-Palmitoyltransferase; Cloning, Molecular; DNA, Complementary; Enzyme Inhibitors; Humans; Isoenzymes; Kinetics; Malonyl Coenzyme A; Mice; Mitochondria, Liver; Mitochondria, Muscle; Molecular Sequence Data; Organ Specificity; Pichia; Rats; Sequence Alignment; Swine

2001
Medium-chain acyl-CoA dehydrogenase (MCAD) mutations identified by MS/MS-based prospective screening of newborns differ from those observed in patients with clinical symptoms: identification and characterization of a new, prevalent mutation that results i
    American journal of human genetics, 2001, Volume: 68, Issue:6

    Topics: Acyl-CoA Dehydrogenase; Acyl-CoA Dehydrogenases; Alleles; Carnitine; Chaperonin 10; Chaperonin 60; DNA Mutational Analysis; Enzyme Stability; Escherichia coli; Exons; Genetic Testing; Haplotypes; Heterozygote; Homozygote; Humans; Infant, Newborn; Mass Spectrometry; Molecular Sequence Data; Mutagenesis, Site-Directed; Mutation, Missense; Polymorphism, Single Nucleotide; Protein Folding; Recombinant Proteins; Reproducibility of Results; Temperature

2001
Impact of the intramitochondrial enzyme organization on fatty acid oxidation.
    Biochemical Society transactions, 2001, Volume: 29, Issue:Pt 2

    Topics: Acyl-CoA Dehydrogenase; Acyl-CoA Dehydrogenase, Long-Chain; Animals; Carnitine; Fatty Acids; Fatty Acids, Volatile; Membrane Proteins; Mitochondria; Oxidation-Reduction; Oxidoreductases; Rats; Substrate Specificity

2001
Design of a pilot study to evaluate tandem mass spectrometry for neonatal screening.
    The Southeast Asian journal of tropical medicine and public health, 1999, Volume: 30 Suppl 2

    Topics: Amino Acids; Carnitine; Humans; Infant, Newborn; Mass Spectrometry; Metabolism, Inborn Errors; Neonatal Screening; Pilot Projects

1999
Molecular and functional characterisation of mild MCAD deficiency.
    Human genetics, 2001, Volume: 108, Issue:5

    Topics: Acyl-CoA Dehydrogenase; Acyl-CoA Dehydrogenases; Carnitine; Consanguinity; DNA Mutational Analysis; Female; Fibroblasts; Genetic Variation; Germany; Heterozygote; Homozygote; Humans; Infant; Lymphocytes; Male; Mutation; Mutation, Missense; Phenylpropionates; Protein Processing, Post-Translational; Turkey

2001
Multiple acyl-CoA-dehydrogenase deficiency (MADD): use of acylcarnitines and fatty acids to monitor the response to dietary treatment.
    Pediatric research, 2001, Volume: 50, Issue:1

    Topics: Acyl-CoA Dehydrogenase; Acyl-CoA Dehydrogenases; Amino Acid Metabolism, Inborn Errors; Carnitine; Diet; Fatty Acids; Female; Humans; Infant, Newborn; Lipid Metabolism, Inborn Errors; Neonatal Screening

2001
Electrospray tandem mass spectrometry for analysis of acylcarnitines in dried postmortem blood specimens collected at autopsy from infants with unexplained cause of death.
    Clinical chemistry, 2001, Volume: 47, Issue:7

    Topics: Autopsy; Bile; Blood Specimen Collection; Canada; Carnitine; Fatty Acids; Humans; Infant; Infant, Newborn; Metabolism, Inborn Errors; Oxidation-Reduction; Paper; Reference Values; Spectrometry, Mass, Electrospray Ionization; Sudden Infant Death; United States

2001
Protection of ischemic hearts perfused with an anion exchange inhibitor, DIDS, is associated with beneficial changes in substrate metabolism.
    Cardiovascular research, 2001, Aug-01, Volume: 51, Issue:2

    Topics: 4,4'-Diisothiocyanostilbene-2,2'-Disulfonic Acid; Adenosine Triphosphate; Animals; Anion Exchange Protein 1, Erythrocyte; Carnitine; Fatty Acids; Glucose; Myocardial Ischemia; Myocardial Reperfusion; Myocardium; Oxidation-Reduction; Perfusion; Phosphocreatine; Phospholipids; Rats; Triglycerides

2001
Hyperinsulinism in short-chain L-3-hydroxyacyl-CoA dehydrogenase deficiency reveals the importance of beta-oxidation in insulin secretion.
    The Journal of clinical investigation, 2001, Volume: 108, Issue:3

    Topics: 3-Hydroxyacyl CoA Dehydrogenases; Amino Acid Sequence; Animals; Base Sequence; Carnitine; DNA Primers; Evolution, Molecular; Fatty Acids; Female; Gene Expression; Homozygote; Humans; Hyperinsulinism; Hypoglycemia; In Vitro Techniques; Infant; Insulin; Insulin Secretion; Models, Biological; Molecular Sequence Data; Oxidation-Reduction; Point Mutation; Recombinant Proteins; Sequence Homology, Amino Acid

2001
A rapid and quantitative mass spectrometry method for determining the concentration of acylcarnitines and aminoacids in amniotic fluid.
    Prenatal diagnosis, 2001, Volume: 21, Issue:7

    Topics: Amino Acids; Amniotic Fluid; Carnitine; Female; Fetal Diseases; Humans; Mass Spectrometry; Metabolic Diseases; Predictive Value of Tests; Pregnancy; Prenatal Diagnosis; Sensitivity and Specificity

2001
Valproate induces in vitro accumulation of long-chain fatty acylcarnitines.
    Molecular genetics and metabolism, 2001, Volume: 73, Issue:4

    Topics: Carnitine; Cell Line; Fatty Acids; Fibroblasts; Gas Chromatography-Mass Spectrometry; Humans; Mitochondria; Mitochondrial Trifunctional Protein; Multienzyme Complexes; Palmitic Acid; Valproic Acid

2001
Evaluation of newborn screening for medium chain acyl-CoA dehydrogenase deficiency in 275 000 babies.
    Archives of disease in childhood. Fetal and neonatal edition, 2001, Volume: 85, Issue:2

    Topics: Acyl-CoA Dehydrogenase; Acyl-CoA Dehydrogenases; Australian Capital Territory; Carnitine; Humans; Infant, Newborn; Mass Spectrometry; Mutation; Neonatal Screening; New South Wales; Prospective Studies; Retrospective Studies

2001
Hepatic carnitine palmitoyltransferase I deficiency: acylcarnitine profiles in blood spots are highly specific.
    Clinical chemistry, 2001, Volume: 47, Issue:10

    Topics: Biomarkers; Blood Specimen Collection; Carnitine; Carnitine O-Palmitoyltransferase; Humans; Infant; Infant, Newborn; Liver; Male; Neonatal Screening; Paper; Sensitivity and Specificity; Spectrometry, Mass, Electrospray Ionization

2001
Oxidation of unsaturated fatty acids by human fibroblasts with very-long-chain acyl-CoA dehydrogenase deficiency: aspects of substrate specificity and correlation with clinical phenotype.
    Clinica chimica acta; international journal of clinical chemistry, 2001, Volume: 312, Issue:1-2

    Topics: Acyl-CoA Dehydrogenase, Long-Chain; Acyl-CoA Dehydrogenases; Adult; Carnitine; Case-Control Studies; Cells, Cultured; Child; Child, Preschool; Fatty Acids, Unsaturated; Female; Fibroblasts; Humans; Infant, Newborn; Isomerism; Lipid Metabolism, Inborn Errors; Oleic Acid; Oleic Acids; Oxidation-Reduction; Substrate Specificity

2001
Gestational, pathologic and biochemical differences between very long-chain acyl-CoA dehydrogenase deficiency and long-chain acyl-CoA dehydrogenase deficiency in the mouse.
    Human molecular genetics, 2001, Sep-15, Volume: 10, Issue:19

    Topics: Acyl-CoA Dehydrogenase, Long-Chain; Animals; Carnitine; Fatty Acids; Female; Humans; Lipid Metabolism, Inborn Errors; Litter Size; Male; Mice; Mice, Inbred C57BL; Mice, Knockout; Mitochondria, Heart; Mitochondria, Liver; Mitochondria, Muscle

2001
Evaluation and application of liquid chromatographic columns coated with 'intelligent' ligands: (I) acylcarnitine column.
    Journal of chromatography. A, 2001, Sep-21, Volume: 929, Issue:1-2

    Topics: Carnitine; Chromatography, Liquid; Reproducibility of Results; Static Electricity

2001
Prenatal diagnosis of multiple acyl-CoA dehydrogenase deficiency: association with elevated alpha-fetoprotein and cystic renal changes.
    Prenatal diagnosis, 2001, Volume: 21, Issue:10

    Topics: Acyl-CoA Dehydrogenase; Acyl-CoA Dehydrogenases; alpha-Fetoproteins; Amniocentesis; Amniotic Fluid; Carnitine; Diagnosis, Differential; Fatty Liver; Female; Fetal Diseases; Fetal Growth Retardation; Gestational Age; Humans; Oligohydramnios; Polycystic Kidney Diseases; Pregnancy; Prenatal Diagnosis; Ultrasonography, Prenatal

2001
Role of intrapartum hypoxia in carnitine nutritional status during the early neonatal period.
    Early human development, 2001, Volume: 65 Suppl

    Topics: Carnitine; Chromatography, High Pressure Liquid; Esters; Fetal Hypoxia; Gestational Age; Humans; Hydrogen-Ion Concentration; Infant, Newborn; Lysine; Methionine; Nutritional Status

2001
Cognitive behavioral therapy and fasting therapy for a patient with chronic fatigue syndrome.
    Internal medicine (Tokyo, Japan), 2001, Volume: 40, Issue:11

    Topics: Adult; Carnitine; Cognitive Behavioral Therapy; Fasting; Fatigue Syndrome, Chronic; Humans; Killer Cells, Natural; Lymphocyte Subsets; Male; Time Factors; Treatment Outcome

2001
Characterization of plasma acylcarnitines in patients under valproate monotherapy using ESI-MS/MS.
    Clinical biochemistry, 2001, Volume: 34, Issue:8

    Topics: Adolescent; Adult; Aged; Anticonvulsants; Carnitine; Female; Humans; Leucine; Male; Middle Aged; Mitochondrial Trifunctional Protein; Multienzyme Complexes; Spectrometry, Mass, Electrospray Ionization; Valerates; Valproic Acid

2001
Acylcarnitine profiles in fibroblasts from patients with respiratory chain defects can resemble those from patients with mitochondrial fatty acid beta-oxidation disorders.
    Metabolism: clinical and experimental, 2002, Volume: 51, Issue:3

    Topics: Carnitine; Cells, Cultured; Fatty Acids; Fibroblasts; Humans; Mitochondria; Mitochondrial Diseases; Oxidation-Reduction; Reference Values

2002
Screening for carnitine palmitoyltransferase II deficiency by tandem mass spectrometry.
    Journal of inherited metabolic disease, 2002, Volume: 25, Issue:1

    Topics: Adolescent; Adult; Aged; Aged, 80 and over; Carnitine; Carnitine O-Palmitoyltransferase; Child; Child, Preschool; Female; Humans; Infant; Male; Mass Screening; Mass Spectrometry; Middle Aged; Palmitoylcarnitine

2002
More on the metabolic autopsy.
    Clinical chemistry, 2002, Volume: 48, Issue:6 Pt 1

    Topics: Amino Acids; Autopsy; Biopsy; Carnitine; Humans; Infant, Newborn; Metabolism, Inborn Errors; Skin; Sudden Infant Death; Urine

2002
Assessment of plasma carnitine concentrations in relation to ceroid lipofuscinosis in Tibetan Terriers.
    American journal of veterinary research, 2002, Volume: 63, Issue:6

    Topics: Age Factors; Animals; Carnitine; Cerebral Cortex; Dog Diseases; Dogs; Female; Male; Microscopy, Electron; Microscopy, Fluorescence; Neuronal Ceroid-Lipofuscinoses; Retina; Sex Factors

2002
Serum acylcarnitines and vitamin B12 deficiency.
    Clinical chemistry, 2002, Volume: 48, Issue:7

    Topics: Aged; Carnitine; Chromatography, High Pressure Liquid; Female; Humans; Male; Mass Spectrometry; Methylmalonic Acid; Middle Aged; Vitamin B 12 Deficiency

2002
Quantitative fibroblast acylcarnitine profiles in mitochondrial fatty acid beta-oxidation defects: phenotype/metabolite correlations.
    Molecular genetics and metabolism, 2002, Volume: 76, Issue:4

    Topics: Acyl-CoA Dehydrogenase; Acyl-CoA Dehydrogenase, Long-Chain; Adolescent; Adult; Carnitine; Child; Child, Preschool; Fatty Acid Desaturases; Fatty Acids; Fibroblasts; Humans; Infant; Infant, Newborn; Metabolism, Inborn Errors; Mitochondria; Oxidation-Reduction; Phenotype

2002
Interactions of acyl carnitines with model membranes: a (13)C-NMR study.
    Journal of lipid research, 2002, Volume: 43, Issue:9

    Topics: Animals; Carnitine; Cell Membrane; Egg Yolk; Hydrogen-Ion Concentration; Lipid Bilayers; Liposomes; Magnetic Resonance Spectroscopy; Models, Biological; Molecular Structure; Solutions; Water

2002
A method for quantitative acylcarnitine profiling in human skin fibroblasts using unlabelled palmitic acid: diagnosis of fatty acid oxidation disorders and differentiation between biochemical phenotypes of MCAD deficiency.
    Biochimica et biophysica acta, 2002, Oct-10, Volume: 1584, Issue:2-3

    Topics: Acyl-CoA Dehydrogenase; Acyl-CoA Dehydrogenases; Carnitine; Cells, Cultured; Fibroblasts; Humans; Infant, Newborn; Lipid Metabolism, Inborn Errors; Mitochondria; Palmitic Acid; Phenotype; Sensitivity and Specificity; Skin; Spectrometry, Mass, Electrospray Ionization

2002
Fat oxidation defect presenting with overwhelming ketonuria.
    Archives of disease in childhood, 2002, Volume: 87, Issue:5

    Topics: Acyl-CoA Dehydrogenase, Long-Chain; Brain Diseases, Metabolic; Carnitine; Female; Humans; Hypoglycemia; Infant; Ketone Bodies; Oxidation-Reduction

2002
Comprehensive screening of urine samples for inborn errors of metabolism by electrospray tandem mass spectrometry.
    Clinical chemistry, 2002, Volume: 48, Issue:11

    Topics: Amino Acids; Carboxylic Acids; Carnitine; Humans; Mass Screening; Metabolism, Inborn Errors; Spectrometry, Mass, Electrospray Ionization

2002
Identification of two novel mutations in OCTN2 from two Saudi patients with systemic carnitine deficiency.
    Journal of inherited metabolic disease, 2002, Volume: 25, Issue:5

    Topics: Carnitine; Carrier Proteins; Child, Preschool; Humans; Infant; Male; Mass Spectrometry; Membrane Proteins; Mutation, Missense; Organic Cation Transport Proteins; Solute Carrier Family 22 Member 5

2002
Acylcarnitine profiles of preterm infants over the first four weeks of life.
    Pediatric research, 2002, Volume: 52, Issue:5

    Topics: Carnitine; Gestational Age; Humans; Infant, Newborn; Infant, Premature; Metabolism, Inborn Errors; Neonatal Screening; Spectrometry, Mass, Electrospray Ionization; Time Factors

2002
Effects of fasting and hypoxic preconditioning on the hypoxic-reoxygenated ventricular strips of the rat heart.
    Journal of physiology and biochemistry, 2002, Volume: 58, Issue:2

    Topics: Acyl Coenzyme A; Analysis of Variance; Animals; Carnitine; Fasting; Heart Ventricles; Hypoxia; Ischemic Preconditioning, Myocardial; Lactic Acid; Myocardial Contraction; Myocardial Reperfusion Injury; Rats; Rats, Wistar

2002
Relative importance of enhanced glucose uptake versus attenuation of long-chain acyl carnitines in protecting ischemic myocardium.
    Coronary artery disease, 2002, Volume: 13, Issue:6

    Topics: Animals; Carnitine; Creatine Kinase; Cytochalasin B; Disease Models, Animal; Glucose; Models, Cardiovascular; Myocardial Contraction; Myocardial Ischemia; Myocardial Reperfusion Injury; Niacin; Oxygen Consumption; Rats; Recovery of Function; Stroke Volume; Vasodilator Agents; Ventricular Pressure

2002
Validation of an ESI-MS/MS screening method for acylcarnitine profiling in urine specimens of neonates, children, adolescents and adults.
    Clinica chimica acta; international journal of clinical chemistry, 2003, Volume: 327, Issue:1-2

    Topics: Adolescent; Adult; Age Factors; Carnitine; Case-Control Studies; Child; Child, Preschool; Humans; Infant; Infant, Newborn; Mass Screening; Metabolism, Inborn Errors; Reproducibility of Results; Sensitivity and Specificity; Spectrometry, Mass, Electrospray Ionization

2003
Neonatal presentation of ventricular tachycardia and a Reye-like syndrome episode associated with disturbed mitochondrial energy metabolism.
    BMC pediatrics, 2002, Dec-30, Volume: 2

    Topics: Biomarkers; Carnitine; DNA, Mitochondrial; Female; Glutarates; Humans; Infant, Newborn; Mitochondrial Myopathies; Mutation; Orotic Acid; Reye Syndrome; Tachycardia, Ventricular

2002
The pattern of urinary acylcarnitines determined by electrospray mass spectrometry: a new tool in the diagnosis of diabetes mellitus.
    Analytical and bioanalytical chemistry, 2003, Volume: 375, Issue:2

    Topics: Adult; Aged; Body Mass Index; Calibration; Carnitine; Diabetes Mellitus; Humans; Middle Aged; Spectrometry, Mass, Electrospray Ionization

2003
Determination of carnitine and acylcarnitines in urine by high-performance liquid chromatography-electrospray ionization ion trap tandem mass spectrometry.
    Journal of chromatography. A, 2003, Jan-17, Volume: 984, Issue:2

    Topics: Carnitine; Chromatography, High Pressure Liquid; Reproducibility of Results; Spectrometry, Mass, Electrospray Ionization

2003
Characteristic acylcarnitine profiles in inherited defects of peroxisome biogenesis: a novel tool for screening diagnosis using tandem mass spectrometry.
    Pediatric research, 2003, Volume: 53, Issue:6

    Topics: Carnitine; Gas Chromatography-Mass Spectrometry; Humans; Mass Spectrometry; Peroxisomal Disorders; Peroxisomes

2003
The frequency of short-chain acyl-CoA dehydrogenase gene variants in the US population and correlation with the C(4)-acylcarnitine concentration in newborn blood spots.
    Molecular genetics and metabolism, 2003, Volume: 78, Issue:4

    Topics: Alleles; Butyryl-CoA Dehydrogenase; Carnitine; Exons; Fatty Acids; Gene Frequency; Genetic Variation; Genetics, Population; Genotype; Heterozygote; Homozygote; Humans; Infant, Newborn; Mass Screening; Mass Spectrometry; Mutation; Phenotype; Scattering, Radiation; Temperature; United States

2003
Expanded newborn screening for inborn errors of metabolism by electrospray ionization-tandem mass spectrometry: results, outcome, and implications.
    Pediatrics, 2003, Volume: 111, Issue:6 Pt 1

    Topics: Acyl-CoA Dehydrogenase; Amino Acid Metabolism, Inborn Errors; Amino Acids; Carnitine; Cohort Studies; Decision Trees; Evidence-Based Medicine; Fatty Acid Desaturases; Follow-Up Studies; Germany; Humans; Infant, Newborn; Infant, Premature; Infant, Premature, Diseases; Metabolism, Inborn Errors; Neonatal Screening; Predictive Value of Tests; Prospective Studies; Sensitivity and Specificity; Spectrometry, Mass, Electrospray Ionization; Treatment Outcome

2003
Effect of supplementation with L-carnitine at a small dose on acylcarnitine profiles in serum and urine and the renal handling of acylcarnitines in a patient with multiple acyl-coenzyme A dehydrogenation defect.
    Journal of chromatography. B, Analytical technologies in the biomedical and life sciences, 2003, Jul-15, Volume: 792, Issue:1

    Topics: Acyl-CoA Dehydrogenase; Carnitine; Humans; Infant, Newborn; Kidney; Male; Metabolism, Inborn Errors; Spectrometry, Mass, Electrospray Ionization

2003
Kinetic analysis of the selectivity of acylcarnitine synthesis in rat mitochondria.
    Lipids, 2003, Volume: 38, Issue:4

    Topics: alpha-Linolenic Acid; Animals; Binding, Competitive; Carnitine; Carnitine O-Palmitoyltransferase; Cattle; Fatty Acids; Fatty Acids, Nonesterified; Kinetics; Male; Mitochondria, Liver; Oleic Acid; Rats; Rats, Sprague-Dawley; Serum Albumin, Bovine

2003
Evaluation of 3-methylcrotonyl-CoA carboxylase deficiency detected by tandem mass spectrometry newborn screening.
    Journal of inherited metabolic disease, 2003, Volume: 26, Issue:1

    Topics: Acids; Carbon-Carbon Ligases; Carnitine; Female; Genetic Testing; Humans; Infant, Newborn; Lymphocytes; Male; Mass Spectrometry; Metabolism, Inborn Errors; Neonatal Screening; North Carolina; Pilot Projects

2003
A novel mitochondrial carnitine-acylcarnitine translocase induced by partial hepatectomy and fasting.
    The Journal of biological chemistry, 2003, Oct-03, Volume: 278, Issue:40

    Topics: 3T3 Cells; Amino Acid Sequence; Animals; Biological Transport; Blotting, Northern; Blotting, Western; Brain; Carnitine; Carnitine Acyltransferases; DNA, Complementary; Escherichia coli; Fatty Acids; Food Deprivation; Genetic Complementation Test; Humans; Immunohistochemistry; Lipid Metabolism; Liver; Mice; Mice, Inbred C57BL; Mitochondria; Mitochondrial Proteins; Molecular Sequence Data; Palmitoylcarnitine; Plasmids; Saccharomyces cerevisiae; Sequence Homology, Amino Acid; Time Factors; Tissue Distribution

2003
Adult presentation of MCAD deficiency revealed by coma and severe arrythmias.
    Intensive care medicine, 2003, Volume: 29, Issue:9

    Topics: Acyl-CoA Dehydrogenase; Adult; Arrhythmias, Cardiac; Carnitine; Coma; Critical Care; Dicarboxylic Acids; Humans; Male; Metabolism, Inborn Errors; Treatment Outcome

2003
What is the role of medium-chain triglycerides in the management of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency?
    Journal of inherited metabolic disease, 2003, Volume: 26, Issue:4

    Topics: 3-Hydroxyacyl CoA Dehydrogenases; Carnitine; Child; Child, Preschool; Fatty Acids, Nonesterified; Food, Formulated; Humans; Metabolism, Inborn Errors; Respiratory Mechanics; Triglycerides

2003
A case of mitochondrial trifunctional protein deficiency diagnosed by acylcarnitine profile and DNA analysis in a dried blood spot of a 4-day-old boy.
    Journal of inherited metabolic disease, 2003, Volume: 26, Issue:4

    Topics: Amino Acid Metabolism, Inborn Errors; Carnitine; Cytosine; DNA Mutational Analysis; Fatal Outcome; Guanine; Humans; Infant, Newborn; Male; Mitochondrial Trifunctional Protein; Multienzyme Complexes; Mutation; Protein Isoforms

2003
LONG-CHAIN CARNITINE ACYLTRANSFERASE AND THE ROLE OF ACYLCARNITINE DERIVATIVES IN THE CATALYTIC INCREASE OF FATTY ACID OXIDATION INDUCED BY CARNITINE.
    Journal of lipid research, 1963, Volume: 4

    Topics: Acyltransferases; Adenosine Triphosphate; Carbon Isotopes; Carnitine; Carnitine Acyltransferases; Chromatography; Coenzyme A; Lipid Metabolism; Metabolism; Mitochondria; Myocardium; Palmitic Acid; Pharmacology; Research; Tritium; Vitamin B Complex

1963
THE FORMATION AND ISOLATION OF LONG-CHAIN ACYL CARNITINES IN MITOCHONDRIA.
    Biochimica et biophysica acta, 1965, Apr-05, Volume: 98

    Topics: Animals; Carbon Isotopes; Carnitine; Cattle; Chromatography; Folic Acid; Metabolism; Mitochondria; Myocardium; Palmitic Acid; Phospholipids; Research; Vitamin B Complex

1965
TWO-DIMENSIONAL THIN-LAYER CHROMATOGRAPHIC ISOLATION OF FATTY ACYL CARNITINES.
    Journal of lipid research, 1965, Volume: 6

    Topics: Carnitine; Chromatography; Chromatography, Thin Layer; Phospholipids; Research; Vitamin B Complex

1965
Defective hepatic mitochondrial respiratory chain in patients with nonalcoholic steatohepatitis.
    Hepatology (Baltimore, Md.), 2003, Volume: 38, Issue:4

    Topics: Adult; Body Mass Index; Carnitine; Electron Transport; Fatty Acids; Fatty Liver; Female; Humans; Insulin Resistance; Male; Middle Aged; Mitochondria, Liver; Oxidation-Reduction; Reactive Oxygen Species

2003
The mitochondrial acetoacetyl-CoA thiolase (T2) deficiency in Japanese patients: urinary organic acid and blood acylcarnitine profiles under stable conditions have subtle abnormalities in T2-deficient patients with some residual T2 activity.
    Journal of inherited metabolic disease, 2003, Volume: 26, Issue:5

    Topics: Acetoacetates; Acetyl-CoA C-Acetyltransferase; Amino Acid Metabolism, Inborn Errors; Carnitine; Humans; Hydroxybutyrates; Infant; Isoleucine; Ketone Bodies; Male; Mitochondria; Mutation

2003
A knock-out mouse model for methylmalonic aciduria resulting in neonatal lethality.
    The Journal of biological chemistry, 2003, Dec-26, Volume: 278, Issue:52

    Topics: Alkyl and Aryl Transferases; Amino Acid Metabolism, Inborn Errors; Animals; Blotting, Southern; Carnitine; Cell Line; Citrates; DNA; Fibroblasts; Genotype; Homozygote; Liver; Methylmalonic Acid; Methylmalonyl-CoA Mutase; Mice; Mice, Knockout; Mice, Transgenic; Models, Genetic; Phenotype; Plasmids; Protein Structure, Tertiary; RNA, Messenger; Time Factors

2003
A comparison of in vitro acylcarnitine profiling methods for the diagnosis of classical and variant short chain acyl-CoA dehydrogenase deficiency.
    Clinica chimica acta; international journal of clinical chemistry, 2003, Volume: 337, Issue:1-2

    Topics: Acyl-CoA Dehydrogenase; Butyric Acid; Butyryl-CoA Dehydrogenase; Carbon Isotopes; Carnitine; Deuterium; DNA Mutational Analysis; Fibroblasts; Heterozygote; Homozygote; Humans; Malonates; Mutation, Missense; Palmitic Acid; Palmitoylcarnitine; Polymorphism, Genetic; Sensitivity and Specificity; Spectrometry, Mass, Electrospray Ionization; Spiro Compounds

2003
Glutaryl-CoA dehydrogenase deficiency: region-specific analysis of organic acids and acylcarnitines in post mortem brain predicts vulnerability of the putamen.
    Neuropediatrics, 2003, Volume: 34, Issue:5

    Topics: Acidosis; Acute Disease; Adolescent; Anticonvulsants; Atrophy; Brain; Carnitine; DNA Mutational Analysis; Fatal Outcome; Gas Chromatography-Mass Spectrometry; Gene Expression; Glutarates; Glutaryl-CoA Dehydrogenase; Humans; Male; Muscle Hypotonia; N-Methylaspartate; Oxidoreductases Acting on CH-CH Group Donors; Point Mutation; Putamen; Spasm; Vigabatrin

2003
Rapid diagnosis of medium chain Acyl Co-A dehydrogenase (MCAD) deficiency in a newborn by liquid chromatography/tandem mass spectrometry.
    Rapid communications in mass spectrometry : RCM, 2003, Volume: 17, Issue:23

    Topics: Acyl-CoA Dehydrogenase; Calibration; Carnitine; Chromatography, High Pressure Liquid; DNA; Humans; Infant, Newborn; Male; Mass Spectrometry; Metabolism, Inborn Errors; Neonatal Screening; Reverse Transcriptase Polymerase Chain Reaction

2003
Respiratory complex II defect in siblings associated with a symptomatic secondary block in fatty acid oxidation.
    Journal of inherited metabolic disease, 2003, Volume: 26, Issue:7

    Topics: Acidosis, Lactic; Acyl-CoA Dehydrogenase; Behavior; Bicarbonates; Carnitine; Cell Line; Fatty Acids; Female; Fibroblasts; Growth; Humans; Infant, Newborn; Lactic Acid; Mitochondrial Diseases; Oxidation-Reduction; Oxidative Phosphorylation; Phenotype

2003
Serum carnitine concentrations correlated to clinical outcome parameters in chronic hemodialysis patients.
    Clinical nutrition (Edinburgh, Scotland), 2004, Volume: 23, Issue:1

    Topics: Age Factors; Blood Urea Nitrogen; Carnitine; Diabetes Complications; Diabetes Mellitus; Female; Humans; Kidney Failure, Chronic; Male; Mannitol; Middle Aged; Parathyroid Hormone; Renal Dialysis; Treatment Outcome; Ventricular Dysfunction, Left

2004
Hepatic expression of malonyl-CoA decarboxylase reverses muscle, liver and whole-animal insulin resistance.
    Nature medicine, 2004, Volume: 10, Issue:3

    Topics: Acyl Coenzyme A; Adenoviridae; Animals; Carboxy-Lyases; Carnitine; Cells, Cultured; Dietary Fats; Hepatocytes; Insulin; Insulin Resistance; Lipid Metabolism; Liver; Male; Muscle, Skeletal; Rats; Rats, Wistar; Triglycerides

2004
Blood acylcarnitine levels in normal newborns and heterozygotes for medium-chain acyl-CoA dehydrogenase deficiency: a relationship between genotype and biochemical phenotype?
    Journal of inherited metabolic disease, 2004, Volume: 27, Issue:1

    Topics: Acyl-CoA Dehydrogenase; Adenine; Carnitine; Diagnosis, Differential; Gene Frequency; Genotype; Guanine; Heterozygote; Humans; Infant, Low Birth Weight; Infant, Newborn; Metabolism, Inborn Errors; Osmolar Concentration; Phenotype

2004
Changes in blood carnitine and acylcarnitine profiles of very long-chain acyl-CoA dehydrogenase-deficient mice subjected to stress.
    European journal of clinical investigation, 2004, Volume: 34, Issue:3

    Topics: Acyl-CoA Dehydrogenase, Long-Chain; Animals; Biomarkers; Blood Glucose; Carnitine; Cold Temperature; Fasting; Mice; Mice, Knockout; Motor Activity; Phenotype; Stress, Physiological

2004
Synthesis of dicarboxylic acylcarnitines.
    Chemistry and physics of lipids, 2004, Volume: 129, Issue:2

    Topics: Carnitine; Deuterium; Dicarboxylic Acids; Magnetic Resonance Spectroscopy; Spectrometry, Mass, Electrospray Ionization

2004
Determination of carnitine and acylcarnitines in plasma by high-performance liquid chromatography/electrospray ionization ion trap tandem mass spectrometry.
    Rapid communications in mass spectrometry : RCM, 2004, Volume: 18, Issue:11

    Topics: Calibration; Carnitine; Chromatography, High Pressure Liquid; Humans; Methylmalonic Acid; Reference Standards; Reproducibility of Results; Sensitivity and Specificity; Spectrometry, Mass, Electrospray Ionization

2004
Homozygosity for a severe novel medium-chain acyl-CoA dehydrogenase (MCAD) mutation IVS3-1G > C that leads to introduction of a premature termination codon by complete missplicing of the MCAD mRNA and is associated with phenotypic diversity ranging from s
    Molecular genetics and metabolism, 2004, Volume: 82, Issue:2

    Topics: Acyl-CoA Dehydrogenase; Adult; Alternative Splicing; Arabs; Base Sequence; Carnitine; Child; Codon, Nonsense; Female; Homozygote; Humans; Infant, Newborn; Male; Mutation; Oxidation-Reduction; Pedigree; Phenotype; RNA, Messenger; Sudden Infant Death

2004
Pseudo-glutarylcarnitinaemia in medium-chain acyl-CoA dehydrogenase deficiency detected by tandem mass spectrometry newborn screening.
    Journal of inherited metabolic disease, 2004, Volume: 27, Issue:4

    Topics: Acetylation; Acyl-CoA Dehydrogenase; Carnitine; False Positive Reactions; Fatty Acids; Gas Chromatography-Mass Spectrometry; Humans; Infant, Newborn; Mass Spectrometry; Neonatal Screening; Oxidation-Reduction; Sensitivity and Specificity

2004
Genetic and biochemical study in a patient with glutaric acidemia type I.
    Journal of the Formosan Medical Association = Taiwan yi zhi, 2004, Volume: 103, Issue:7

    Topics: Amino Acid Metabolism, Inborn Errors; Carnitine; Child, Preschool; Glutarates; Glutaryl-CoA Dehydrogenase; Humans; Male; Mutation; Oxidoreductases Acting on CH-CH Group Donors

2004
Dobutamine alters carnitine metabolism in the neonatal piglet heart.
    Canadian journal of physiology and pharmacology, 2004, Volume: 82, Issue:7

    Topics: Animals; Animals, Newborn; Cardiotonic Agents; Carnitine; Coronary Circulation; Dobutamine; Dose-Response Relationship, Drug; Heart Rate; In Vitro Techniques; Myocardium; Swine; Ventricular Function, Left; Ventricular Pressure

2004
Isobutyryl-CoA dehydrogenase deficiency: isobutyrylglycinuria and ACAD8 gene mutations in two infants.
    Journal of inherited metabolic disease, 2004, Volume: 27, Issue:6

    Topics: Acyl-CoA Dehydrogenases; Carnitine; Female; Frameshift Mutation; Humans; Infant, Newborn; Male; Mass Spectrometry; Mutation; Mutation, Missense; Neonatal Screening; Oxidoreductases Acting on CH-CH Group Donors

2004
Peroxisome proliferator activated receptor delta (PPARdelta) agonist but not PPARalpha corrects carnitine palmitoyl transferase 2 deficiency in human muscle cells.
    The Journal of clinical endocrinology and metabolism, 2005, Volume: 90, Issue:3

    Topics: Adult; Bezafibrate; Butyrates; Carnitine; Carnitine O-Palmitoyltransferase; Cells, Cultured; Dose-Response Relationship, Drug; Enzyme Activation; Gene Expression; Humans; Hypolipidemic Agents; Muscle Fibers, Skeletal; Palmitic Acid; Phenylurea Compounds; Point Mutation; PPAR gamma; Thiazoles; Tritium

2005
Quantitation of long-chain acylcarnitines by HPLC/fluorescence detection: application to plasma and tissue specimens from patients with carnitine palmitoyltransferase-II deficiency.
    Clinica chimica acta; international journal of clinical chemistry, 2005, Volume: 352, Issue:1-2

    Topics: Carnitine; Carnitine O-Palmitoyltransferase; Chromatography, High Pressure Liquid; Humans; Lipid Metabolism, Inborn Errors; Sensitivity and Specificity; Spectrometry, Fluorescence

2005
Filter paper cards contaminated with EMLA cream produce artefacts on acylcarnitine analysis.
    Journal of inherited metabolic disease, 2004, Volume: 27, Issue:5

    Topics: Carnitine; Chemistry, Clinical; Humans; Lidocaine; Prilocaine; Spectrometry, Mass, Electrospray Ionization; Time Factors

2004
Age-related variations in acylcarnitine and free carnitine concentrations measured by tandem mass spectrometry.
    Clinical chemistry, 2005, Volume: 51, Issue:4

    Topics: Adolescent; Adult; Age Factors; Blood Specimen Collection; Carnitine; Child; Child, Preschool; Female; Fetal Blood; Humans; Infant; Infant, Newborn; Male; Mass Spectrometry; Middle Aged; Reference Values; Sex Factors

2005
Serum carnitine levels in patients with homozygous beta thalassemia: a possible new role for carnitine?
    European journal of pediatrics, 2005, Volume: 164, Issue:3

    Topics: Adolescent; Adult; beta-Thalassemia; Carnitine; Case-Control Studies; Child; Child, Preschool; Fatty Acids; Female; Homozygote; Humans; Male; Muscle Weakness; Surveys and Questionnaires

2005
Strategy for the isolation, derivatization, chromatographic separation, and detection of carnitine and acylcarnitines.
    Analytical chemistry, 2005, Mar-01, Volume: 77, Issue:5

    Topics: Acetonitriles; Acetophenones; Acetylcarnitine; Animals; Blood Chemical Analysis; Carnitine; Chemical Precipitation; Chromatography, High Pressure Liquid; Chromatography, Ion Exchange; Humans; Liver; Male; Mass Spectrometry; Methanol; Molecular Structure; Palmitoylcarnitine; Rats; Rats, Sprague-Dawley; Reproducibility of Results; Serum Albumin; Silica Gel; Silicon Dioxide; Solid Phase Extraction

2005
Tissue carnitine homeostasis in very-long-chain acyl-CoA dehydrogenase-deficient mice.
    Pediatric research, 2005, Volume: 57, Issue:6

    Topics: Acyl-CoA Dehydrogenase, Long-Chain; Animals; Bile; Carnitine; Disease Models, Animal; Homeostasis; Humans; Lipid Metabolism, Inborn Errors; Liver; Mice; Mice, Knockout; Muscle, Skeletal; Phenotype

2005
Abnormal concentrations of esterified carnitine in bile: a feature of pediatric acute liver failure with poor prognosis.
    Hepatology (Baltimore, Md.), 2005, Volume: 41, Issue:4

    Topics: Bile; Carnitine; Case-Control Studies; Child; Esterification; Humans; Liver; Liver Failure, Acute; Liver Transplantation; Osmolar Concentration; Retrospective Studies; Risk

2005
Accumulation of long-chain acylcarnitine and 3-hydroxy acylcarnitine molecular species in diabetic myocardium: identification of alterations in mitochondrial fatty acid processing in diabetic myocardium by shotgun lipidomics.
    Biochemistry, 2005, Apr-05, Volume: 44, Issue:13

    Topics: Animals; Base Sequence; Cardiomyopathies; Carnitine; Cricetinae; Diabetes Mellitus, Experimental; DNA; Group VI Phospholipases A2; Hydroxylation; Insulin; Male; Mice; Mice, Transgenic; Myocardial Ischemia; Myocardium; Phospholipases A; Rats; Rats, Sprague-Dawley; RNA, Messenger; Spectrometry, Mass, Electrospray Ionization

2005
Lack of correlation between fatty acid oxidation disorders and haemolysis, elevated liver enzymes, low platelets (HELLP) syndrome?
    Acta paediatrica (Oslo, Norway : 1992), 2005, Volume: 94, Issue:1

    Topics: 3-Hydroxyacyl CoA Dehydrogenases; Adult; Carnitine; Case-Control Studies; DNA Mutational Analysis; Female; HELLP Syndrome; Humans; Infant, Newborn; Male; Metabolism, Inborn Errors; Pregnancy; Retrospective Studies; Risk Factors

2005
Mitochondrial glycerol-3-phosphate acyltransferase-1 is essential in liver for the metabolism of excess acyl-CoAs.
    The Journal of biological chemistry, 2005, Jul-08, Volume: 280, Issue:27

    Topics: Animals; Carnitine; Coenzyme A; Dietary Fats; Dietary Sucrose; Fatty Liver; Female; Glycerol-3-Phosphate O-Acyltransferase; Insulin Resistance; Ketones; Lipids; Liver; Lysophospholipids; Male; Mice; Mice, Inbred C57BL; Mice, Knockout; Mitochondria; Obesity; Oxidation-Reduction; Triglycerides; Weight Gain

2005
Effect of methanol on endogenous and exogenous carnitine levels in rat plasma.
    Archives of toxicology, 2005, Volume: 79, Issue:10

    Topics: Alcoholic Intoxication; Animals; Area Under Curve; Carnitine; Dose-Response Relationship, Drug; Half-Life; Lethal Dose 50; Male; Metabolic Clearance Rate; Methanol; Random Allocation; Rats; Rats, Sprague-Dawley; Survival Analysis

2005
In vitro correction of medium chain acyl CoA dehydrogenase deficiency with a recombinant adenoviral vector.
    Molecular genetics and metabolism, 2005, Volume: 85, Issue:2

    Topics: Acyl-CoA Dehydrogenase; Adenoviridae; Animals; Carnitine; Cattle; Cell Line; Cytomegalovirus; Fibroblasts; Gene Transfer Techniques; Genes, Immediate-Early; Genetic Vectors; Humans; Mass Spectrometry; Promoter Regions, Genetic; RNA 3' Polyadenylation Signals

2005
Biochemical, clinical and molecular findings in LCHAD and general mitochondrial trifunctional protein deficiency.
    Journal of inherited metabolic disease, 2005, Volume: 28, Issue:4

    Topics: Acyl-CoA Dehydrogenase, Long-Chain; Cardiomyopathies; Carnitine; Exons; Fatty Acids; Fibroblasts; Homozygote; Humans; Lipid Metabolism, Inborn Errors; Male; Mitochondria; Mitochondrial Trifunctional Protein; Multienzyme Complexes; Mutation; Phenotype; Polyneuropathies; Prognosis; Rhabdomyolysis

2005
Normal acylcarnitine levels during confirmation of abnormal newborn screening in long-chain fatty acid oxidation defects.
    Journal of inherited metabolic disease, 2005, Volume: 28, Issue:4

    Topics: Acyl-CoA Dehydrogenase, Long-Chain; Carnitine; Genotype; Humans; Infant, Newborn; Lipid Metabolism, Inborn Errors; Male; Mass Spectrometry; Mitochondrial Trifunctional Protein; Multienzyme Complexes; Mutation; Neonatal Screening; Oxygen; Time Factors

2005
Newborns with C8-acylcarnitine level over the 90th centile have an increased frequency of the common MCAD 985A>G mutation.
    Journal of inherited metabolic disease, 2005, Volume: 28, Issue:4

    Topics: Acyl-CoA Dehydrogenase; Alleles; Carnitine; DNA Mutational Analysis; Fatty Acids; Heterozygote; Humans; Infant, Newborn; Lipid Metabolism, Inborn Errors; Mass Spectrometry; Mutation; Neonatal Screening; Oxygen; Spectrometry, Mass, Electrospray Ionization

2005
Ontogeny and kinetics of carnitine palmitoyltransferase in liver and skeletal muscle of the domestic felid (Felis domestica).
    The Journal of nutritional biochemistry, 2005, Volume: 16, Issue:6

    Topics: Animals; Carnitine; Carnitine O-Palmitoyltransferase; Cats; Kinetics; Liver; Muscle, Skeletal

2005
Plasma and urinary carnitine and acylcarnitines in chronic fatigue syndrome.
    Clinica chimica acta; international journal of clinical chemistry, 2005, Volume: 360, Issue:1-2

    Topics: Adult; Aged; Aged, 80 and over; Arthritis, Rheumatoid; Carnitine; Case-Control Studies; Depression; Fatigue Syndrome, Chronic; Female; Humans; Male; Middle Aged; Muscle Fatigue

2005
Pregnancy increases urinary loss of carnitine and reduces plasma carnitine in Korean women.
    The British journal of nutrition, 2005, Volume: 93, Issue:5

    Topics: Adult; Analysis of Variance; Birth Weight; Carnitine; Case-Control Studies; Cholesterol; Female; Fetal Blood; Humans; Infant, Newborn; Korea; Lipids; Pregnancy; Pregnancy Trimesters; Triglycerides

2005
Carnitine levels in valproic acid-treated psychiatric patients: a cross-sectional study.
    The Journal of clinical psychiatry, 2005, Volume: 66, Issue:5

    Topics: Adult; Aged; Amylases; Anticonvulsants; Carnitine; Cross-Sectional Studies; Female; Humans; Liver Function Tests; Male; Mental Disorders; Middle Aged; Valproic Acid

2005
Acute valproate poisoning: pharmacokinetics, alteration in fatty acid metabolism, and changes during therapy.
    Journal of clinical psychopharmacology, 2005, Volume: 25, Issue:4

    Topics: Anticonvulsants; Brain Edema; Carnitine; Drug Overdose; Fatty Acids; Female; Humans; Hyperammonemia; Male; Mitochondria; Oxidation-Reduction; Pancreatitis; Renal Dialysis; Valproic Acid

2005
Rapid measurement of plasma acylcarnitines by liquid chromatography-tandem mass spectrometry without derivatization.
    Clinica chimica acta; international journal of clinical chemistry, 2005, Volume: 358, Issue:1-2

    Topics: Acylation; Carnitine; Chromatography, Liquid; Mass Spectrometry; Sensitivity and Specificity; Time Factors

2005
Bezafibrate increases very-long-chain acyl-CoA dehydrogenase protein and mRNA expression in deficient fibroblasts and is a potential therapy for fatty acid oxidation disorders.
    Human molecular genetics, 2005, Sep-15, Volume: 14, Issue:18

    Topics: Acyl-CoA Dehydrogenase, Long-Chain; Bezafibrate; Blotting, Western; Carnitine; DNA Primers; Dose-Response Relationship, Drug; Fibroblasts; Gene Expression Regulation, Enzymologic; Humans; Lipid Metabolism, Inborn Errors; Mitochondria; Mutation, Missense; Palmitates; Peroxisome Proliferator-Activated Receptors; Reverse Transcriptase Polymerase Chain Reaction; RNA, Messenger

2005
Novel metabolic and molecular findings in hepatic carnitine palmitoyltransferase I deficiency.
    Molecular genetics and metabolism, 2005, Volume: 86, Issue:3

    Topics: Adult; Carnitine; Carnitine O-Palmitoyltransferase; Child, Preschool; Dicarboxylic Acids; Female; Glutarates; Humans; Infant; Male; Mutation; Palmitates

2005
Impact of haemodialysis on individual endogenous plasma acylcarnitine concentrations in end-stage renal disease.
    Annals of clinical biochemistry, 2005, Volume: 42, Issue:Pt 5

    Topics: Adolescent; Adult; Aged; Carnitine; Female; Humans; Kidney Failure, Chronic; Male; Middle Aged; Renal Dialysis; Spectrometry, Mass, Electrospray Ionization

2005
Quantitative acylcarnitine profiling in peripheral blood mononuclear cells using in vitro loading with palmitic and 2-oxoadipic acids: biochemical confirmation of fatty acid oxidation and organic acid disorders.
    Pediatric research, 2005, Volume: 58, Issue:5

    Topics: Adipates; Carnitine; Child; Child, Preschool; Female; Humans; Infant; Male; Mass Spectrometry; Metabolism, Inborn Errors; Monocytes; Palmitic Acid

2005
Acidified acetonitrile and methanol extractions for quantitative analysis of acylcarnitines in plasma by stable isotope dilution tandem mass spectrometry.
    Journal of chromatography. B, Analytical technologies in the biomedical and life sciences, 2005, Dec-05, Volume: 827, Issue:2

    Topics: Acetonitriles; Carnitine; Chemical Fractionation; Glutarates; Humans; Isotope Labeling; Mass Spectrometry; Metabolism, Inborn Errors; Methanol; Reproducibility of Results

2005
Modified and improved method for the measurement of plasma acylcarnitines by online extraction coupled to liquid chromatography-tandem mass spectrometry without derivatization.
    Clinica chimica acta; international journal of clinical chemistry, 2006, Volume: 365, Issue:1-2

    Topics: Carnitine; Chromatography, Liquid; Humans; Mass Spectrometry; Reproducibility of Results

2006
Effect of L-carnitine on the kinetics of carnitine, acylcarnitines and butyrobetaine in long-term haemodialysis.
    Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association, 2006, Volume: 21, Issue:2

    Topics: Adult; Aged; Betaine; Carnitine; Female; Humans; Male; Middle Aged; Renal Dialysis; Time Factors

2006
Medium-chain acyl-CoA dehydrogenase deficiency: genotype-biochemical phenotype correlations.
    Molecular genetics and metabolism, 2006, Volume: 87, Issue:1

    Topics: Acyl-CoA Dehydrogenase; Carnitine; DNA Mutational Analysis; Fibroblasts; Genetic Testing; Genetic Variation; Genotype; Glycine; Homozygote; Humans; Infant, Newborn; Mass Spectrometry; Phenotype; Statistics as Topic

2006
Differentiation of long-chain fatty acid oxidation disorders using alternative precursors and acylcarnitine profiling in fibroblasts.
    Molecular genetics and metabolism, 2006, Volume: 87, Issue:1

    Topics: 3-Hydroxyacyl CoA Dehydrogenases; Acetyl-CoA C-Acyltransferase; Acyl-CoA Dehydrogenase, Long-Chain; Adolescent; Carbon-Carbon Double Bond Isomerases; Carnitine; Cells, Cultured; Clinical Enzyme Tests; Diagnosis, Differential; DNA, Complementary; Enoyl-CoA Hydratase; Fatty Acids; Fibroblasts; Genetic Testing; Humans; Infant, Newborn; Lipid Metabolism, Inborn Errors; Mitochondrial Trifunctional Protein; Multienzyme Complexes; Oxidation-Reduction; Racemases and Epimerases

2006
Measurement of stable isotopic enrichment and concentration of long-chain fatty acyl-carnitines in tissue by HPLC-MS.
    Journal of lipid research, 2006, Volume: 47, Issue:2

    Topics: Animals; Carbon Isotopes; Carnitine; Chromatography, High Pressure Liquid; Deuterium; Fluorocarbons; Male; Methods; Muscle, Skeletal; Palmitic Acid; Palmitoylcarnitine; Rabbits; Reference Standards; Reproducibility of Results; Spectrometry, Mass, Electrospray Ionization

2006
Carnitine treatment inhibits increases in cerebral carnitine esters and glutamate detected by mass spectrometry after hypoxia-ischemia in newborn rats.
    Stroke, 2006, Volume: 37, Issue:2

    Topics: Animals; Animals, Newborn; Anions; Brain; Cardiolipins; Carnitine; Carnitine O-Acetyltransferase; Glutamates; Glutamic Acid; Glycine; Hypoxia; Hypoxia-Ischemia, Brain; Immunohistochemistry; Lipid Metabolism; Mass Spectrometry; Microscopy, Fluorescence; Mitochondria; Phospholipids; Rats; Rats, Wistar; Superoxides; Time Factors; Vitamin B Complex

2006
Bile acylcarnitine profiles in pediatric liver disease do not interfere with the diagnosis of long-chain fatty acid oxidation defects.
    Clinica chimica acta; international journal of clinical chemistry, 2006, Volume: 367, Issue:1-2

    Topics: Bile; Biopsy; Carnitine; Fatty Acids; Humans; Infant; Liver Diseases; Oxidation-Reduction

2006
Rhabdomyolysis in early-onset very long-chain acyl-CoA dehydrogenase deficiency despite normal glucose after fasting.
    Journal of inherited metabolic disease, 2005, Volume: 28, Issue:6

    Topics: Acyl-CoA Dehydrogenase, Long-Chain; Blood Glucose; Carnitine; Fasting; Glucose; Humans; Hypoglycemia; Infant; Metabolism, Inborn Errors; Rhabdomyolysis

2005
Glucose and leucine kinetics in idiopathic ketotic hypoglycaemia.
    Archives of disease in childhood, 2006, Volume: 91, Issue:6

    Topics: Alanine; Amino Acids, Branched-Chain; Basal Metabolism; Calorimetry, Indirect; Carnitine; Case-Control Studies; Child; Child, Preschool; Fasting; Female; Glucose; Humans; Hypoglycemia; Infant; Insulin; Ketosis; Leucine; Male

2006
Carnitine concentrations in term and preterm newborns at birth and during the first days of life.
    Prague medical report, 2005, Volume: 106, Issue:3

    Topics: Carnitine; Female; Fetal Blood; Humans; Infant, Newborn; Infant, Premature; Male

2005
Two newborns with nutritional vitamin B12 deficiency: challenges in newborn screening for vitamin B12 deficiency.
    Haematologica, 2005, Volume: 90, Issue:12 Suppl

    Topics: Acyl-CoA Dehydrogenase, Long-Chain; Anemia, Pernicious; Autoimmune Diseases; Biomarkers; Carnitine; Citrates; Cystathionine; False Negative Reactions; Female; Gastric Bypass; Heterozygote; Homocysteine; Humans; Hydroxocobalamin; Infant Food; Infant, Newborn; Malabsorption Syndromes; Male; Mass Spectrometry; Maternal-Fetal Exchange; Methylmalonic Acid; Neonatal Screening; Pregnancy; Pregnancy Complications; Vitamin B 12 Deficiency

2005
So doctor, what exactly is wrong with my muscles? Glutaric aciduria type II presenting in a teenager.
    Neuromuscular disorders : NMD, 2006, Volume: 16, Issue:4

    Topics: Acyl-CoA Dehydrogenase; Adolescent; Carnitine; DNA Mutational Analysis; Electron-Transferring Flavoproteins; Female; Glutarates; Humans; Iron-Sulfur Proteins; Metabolism, Inborn Errors; Muscular Diseases; Oxidoreductases Acting on CH-NH Group Donors

2006
Functional expression of the organic cation/carnitine transporter 2 in rat astrocytes.
    Journal of neurochemistry, 2006, Volume: 97, Issue:2

    Topics: Animals; Astrocytes; Blotting, Western; Carnitine; Cation Transport Proteins; Cells, Cultured; Dose-Response Relationship, Drug; Drug Interactions; Gene Expression; Glial Fibrillary Acidic Protein; Organic Cation Transport Proteins; Rats; Rats, Sprague-Dawley; RNA, Messenger; RNA, Small Interfering; Solute Carrier Family 22 Member 5; Transfection; Tritium

2006
Relationship between serum carnitine, acylcarnitines, and renal function in patients with chronic renal disease.
    Journal of renal nutrition : the official journal of the Council on Renal Nutrition of the National Kidney Foundation, 2006, Volume: 16, Issue:2

    Topics: Adult; Carnitine; Cross-Sectional Studies; Female; Glomerular Filtration Rate; Humans; Kidney; Kidney Failure, Chronic; Male; Middle Aged

2006
Intracerebral accumulation of glutaric and 3-hydroxyglutaric acids secondary to limited flux across the blood-brain barrier constitute a biochemical risk factor for neurodegeneration in glutaryl-CoA dehydrogenase deficiency.
    Journal of neurochemistry, 2006, Volume: 97, Issue:3

    Topics: Amino Acids; Animals; Biological Transport; Blood-Brain Barrier; Blotting, Western; Brain; Carnitine; Cells, Cultured; Dicarboxylic Acids; Disease Models, Animal; Endothelial Cells; Glucose; Glutarates; Glutaryl-CoA Dehydrogenase; Heart; Liver; Mice; Mice, Inbred C57BL; Mice, Knockout; Models, Biological; Muscles; Neurodegenerative Diseases; Risk Factors; Statistics, Nonparametric; Swine; Time Factors; Tissue Distribution

2006
Refining the diagnosis of mitochondrial HMG-CoA synthase deficiency.
    Journal of inherited metabolic disease, 2006, Volume: 29, Issue:1

    Topics: Carnitine; DNA Mutational Analysis; Genotype; Heterozygote; Humans; Hydroxymethylglutaryl-CoA Synthase; Infant; Male; Metabolism, Inborn Errors; Mitochondrial Diseases; Mutation

2006
A monotopic membrane protein goes solo.
    Structure (London, England : 1993), 2006, Volume: 14, Issue:4

    Topics: Animals; Binding Sites; Carnitine; Carnitine O-Palmitoyltransferase; Catalysis; Cell Membrane; Coenzyme A; Crystallography, X-Ray; Escherichia coli; Fatty Acids; Lipid Bilayers; Molecular Conformation; Protein Binding; Protein Conformation; Protein Structure, Tertiary; Rats; Recombinant Proteins; Solubility; Substrate Specificity

2006
Time course of acylcarnitine elevation in neonatal intrahepatic cholestasis caused by citrin deficiency.
    Journal of inherited metabolic disease, 2006, Volume: 29, Issue:4

    Topics: Alanine; Amino Acid Metabolism, Inborn Errors; Calcium-Binding Proteins; Carnitine; Cholestasis, Intrahepatic; Humans; Infant; Infant, Newborn; Mass Spectrometry; Membrane Transport Proteins; Models, Biological; Neonatal Screening; Organic Anion Transporters; Time Factors

2006
Influence of hematocrit and localisation of punch in dried blood spots on levels of amino acids and acylcarnitines measured by tandem mass spectrometry.
    Clinica chimica acta; international journal of clinical chemistry, 2006, Volume: 373, Issue:1-2

    Topics: Amino Acids; Blood Specimen Collection; Blood Stains; Carnitine; Glycine; Hematocrit; Humans; Sensitivity and Specificity; Tandem Mass Spectrometry

2006
Short-term effects of nocturnal haemodialysis on carnitine metabolism.
    Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association, 2006, Volume: 21, Issue:9

    Topics: Adult; Biological Factors; Carnitine; Circadian Rhythm; Colorimetry; Disease Progression; Female; Follow-Up Studies; Humans; Kidney Failure, Chronic; Male; Middle Aged; Prognosis; Prospective Studies; Renal Dialysis; Time Factors

2006
Pitfalls of neonatal screening for very-long-chain acyl-CoA dehydrogenase deficiency using tandem mass spectrometry.
    The Journal of pediatrics, 2006, Volume: 149, Issue:1

    Topics: Acyl-CoA Dehydrogenase, Long-Chain; Carnitine; Child, Preschool; Female; Genetic Carrier Screening; Humans; Infant, Newborn; Metabolism, Inborn Errors; Mutation, Missense; Neonatal Screening; Spectrometry, Mass, Electrospray Ionization

2006
Effect of temperature on the stability of long-chain acylcarnitines in human blood prior to plasma separation.
    Clinica chimica acta; international journal of clinical chemistry, 2007, Volume: 375, Issue:1-2

    Topics: Adult; Blood Preservation; Carnitine; Clinical Laboratory Techniques; Female; Humans; Male; Middle Aged; Temperature

2007
Carnitine transporter defect: diagnosis in asymptomatic adult women following analysis of acylcarnitines in their newborn infants.
    Journal of inherited metabolic disease, 2006, Volume: 29, Issue:5

    Topics: Adult; Carnitine; Consanguinity; Female; Fetal Blood; Humans; Infant, Newborn; Male; Mass Spectrometry; Metabolism, Inborn Errors; Mothers; Organic Cation Transport Proteins; Oxygen; Solute Carrier Family 22 Member 5

2006
Picolinyl ester fragmentation mechanism studies with application to the identification of acylcarnitine acyl groups following transesterification.
    Journal of the American Society for Mass Spectrometry, 2006, Volume: 17, Issue:11

    Topics: Acylation; Carnitine; Esters; Gas Chromatography-Mass Spectrometry; Picolinic Acids; Spectrometry, Mass, Electrospray Ionization

2006
Prolonged moderate-intensity exercise without and with L-carnitine supplementation in patients with MCAD deficiency.
    Journal of inherited metabolic disease, 2006, Volume: 29, Issue:5

    Topics: Acyl-CoA Dehydrogenase; Adolescent; Adult; Amino Acid Metabolism, Inborn Errors; Carnitine; Child; Dietary Supplements; Exercise; Fatty Acids; Female; Humans; Male; Time Factors

2006
Total carnitine and acylated carnitine ratio: relationship of free carnitine with lipid parameters in pediatric dialysis patients.
    Advances in peritoneal dialysis. Conference on Peritoneal Dialysis, 2006, Volume: 22

    Topics: Adolescent; Body Height; Body Weight; Carnitine; Child; Child, Preschool; Female; Humans; Infant; Lipids; Male; Peritoneal Dialysis; Renal Dialysis

2006
Frequency of low carnitine levels in children on dialysis.
    Advances in peritoneal dialysis. Conference on Peritoneal Dialysis, 2006, Volume: 22

    Topics: Adolescent; Carnitine; Child; Female; Humans; Male; Peritoneal Dialysis; Renal Dialysis

2006
Normal muscle respiratory chain enzymes can complicate mitochondrial disease diagnosis.
    Pediatric neurology, 2006, Volume: 35, Issue:4

    Topics: Acidosis, Lactic; Atrophy; Biopsy; Blotting, Western; Carnitine; Central Nervous System Cysts; Cerebral Ventricles; Diagnosis, Differential; DNA Mutational Analysis; Electron Transport Complex I; Female; Fibroblasts; Frontal Lobe; Humans; Infant, Newborn; Magnetic Resonance Imaging; Mitochondrial Diseases; Muscle, Skeletal; Nerve Fibers, Myelinated; Point Mutation

2006
Studies on the critical micellar concentration and phase transitions of stearoylcarnitine.
    Bioscience reports, 2006, Volume: 26, Issue:6

    Topics: Biophysics; Calorimetry, Differential Scanning; Carnitine; Hydrogen-Ion Concentration; Micelles; Models, Molecular; Spectrometry, Fluorescence; Stearic Acids; Temperature; Water

2006
Late-onset multiple acyl-CoA dehydrogenase deficiency: a frequently missed diagnosis?
    Neurology, 2006, Oct-24, Volume: 67, Issue:8

    Topics: Acyl-CoA Dehydrogenase; Carnitine; Diagnostic Errors; Electron-Transferring Flavoproteins; Heterozygote; Humans; Iron-Sulfur Proteins; Male; Mass Spectrometry; Metabolism, Inborn Errors; Middle Aged; Muscular Diseases; Mutation; Oxidoreductases Acting on CH-NH Group Donors

2006
Tandem mass neonatal screening in Taiwan--report from one center.
    Journal of the Formosan Medical Association = Taiwan yi zhi, 2006, Volume: 105, Issue:11

    Topics: Amino Acids; Carnitine; Female; Humans; Infant, Newborn; Male; Mass Spectrometry; Metabolism, Inborn Errors; Neonatal Screening; Taiwan

2006
ENU mutagenesis identifies mice with cardiac fibrosis and hepatic steatosis caused by a mutation in the mitochondrial trifunctional protein beta-subunit.
    Human molecular genetics, 2006, Dec-15, Volume: 15, Issue:24

    Topics: Adipose Tissue; Animals; Base Sequence; Blotting, Western; Carnitine; Chromosome Mapping; Ethylnitrosourea; Fatty Liver; Female; Fibrosis; Lipid Metabolism Disorders; Male; Mice; Mice, Inbred C57BL; Mitochondrial Trifunctional Protein; Mitochondrial Trifunctional Protein, alpha Subunit; Mitochondrial Trifunctional Protein, beta Subunit; Multienzyme Complexes; Mutagenesis; Myocardium; Point Mutation; Polymerase Chain Reaction; Pregnancy; Tandem Mass Spectrometry

2006
Identification of novel mutations of the HADHA and HADHB genes in patients with mitochondrial trifunctional protein deficiency.
    International journal of molecular medicine, 2007, Volume: 19, Issue:1

    Topics: Amino Acid Metabolism, Inborn Errors; Amino Acid Sequence; Base Sequence; Carnitine; Child; Child, Preschool; DNA Mutational Analysis; Female; Humans; Infant, Newborn; Male; Mitochondrial Trifunctional Protein; Mitochondrial Trifunctional Protein, alpha Subunit; Mitochondrial Trifunctional Protein, beta Subunit; Molecular Sequence Data; Multienzyme Complexes; Mutation; Sequence Homology, Amino Acid; Tandem Mass Spectrometry

2007
Effects of valproate on acylcarnitines in children with epilepsy using ESI-MS/MS.
    Epilepsia, 2007, Volume: 48, Issue:1

    Topics: Adolescent; Anticonvulsants; Carbamazepine; Carnitine; Chemical and Drug Induced Liver Injury; Child; Child, Preschool; Cross-Sectional Studies; Epilepsy; Female; Humans; Infant; Longitudinal Studies; Male; Spectrometry, Mass, Electrospray Ionization; Tandem Mass Spectrometry; Valproic Acid

2007
Long-term stability of amino acids and acylcarnitines in dried blood spots.
    Clinical chemistry, 2007, Volume: 53, Issue:4

    Topics: Amino Acids; Blood Specimen Collection; Carnitine; Humans; Infant, Newborn; Linear Models; Metabolism, Inborn Errors; Models, Biological; Neonatal Screening; Time Factors

2007
Simultaneous quantification of acylcarnitine isomers containing dicarboxylic acylcarnitines in human serum and urine by high-performance liquid chromatography/electrospray ionization tandem mass spectrometry.
    Rapid communications in mass spectrometry : RCM, 2007, Volume: 21, Issue:5

    Topics: Adult; Carnitine; Child, Preschool; Female; Humans; Isomerism; Male; Metabolism, Inborn Errors; Methylmalonic Acid; Methylmalonyl-CoA Mutase; Spectrometry, Mass, Electrospray Ionization

2007
ESI-MS/MS study of acylcarnitine profiles in urine from patients with organic acidemias and fatty acid oxidation disorders.
    Journal of chromatography. B, Analytical technologies in the biomedical and life sciences, 2007, Volume: 855, Issue:1

    Topics: Carnitine; Fatty Acids; Humans; Infant; Lipid Metabolism, Inborn Errors; Metabolism, Inborn Errors; Methylmalonic Acid; Oxidation-Reduction; Propionates; Spectrometry, Mass, Electrospray Ionization; Tandem Mass Spectrometry

2007
Development of a newborn screening follow-up algorithm for the diagnosis of isobutyryl-CoA dehydrogenase deficiency.
    Genetics in medicine : official journal of the American College of Medical Genetics, 2007, Volume: 9, Issue:2

    Topics: Acyl-CoA Dehydrogenases; Algorithms; Carnitine; Diagnosis, Differential; Genetic Testing; Humans; Infant, Newborn; Oxidoreductases Acting on CH-CH Group Donors; United States

2007
Carnitine revisited: potential use as adjunctive treatment in diabetes.
    Diabetologia, 2007, Volume: 50, Issue:4

    Topics: Animals; Calorimetry; Carnitine; Diabetes Mellitus, Experimental; Fatty Acids; Glucose Tolerance Test; Glycerol; Insulin Resistance; Male; Mass Spectrometry; Mice; Mice, Obese; Pyruvate Dehydrogenase (Lipoamide); Vitamin B Complex

2007
2-methylbutyryl-CoA dehydrogenase deficiency in Hmong infants identified by expanded newborn screen.
    WMJ : official publication of the State Medical Society of Wisconsin, 2007, Volume: 106, Issue:1

    Topics: Amino Acid Metabolism, Inborn Errors; Carnitine; Ethnicity; Humans; Infant, Newborn; Mass Spectrometry; Neonatal Screening; Oxidoreductases Acting on CH-CH Group Donors; Vietnam; Wisconsin

2007
Serum carnitine, triglyceride and cholesterol profiles in Korean neonates.
    The British journal of nutrition, 2007, Volume: 98, Issue:2

    Topics: Birth Weight; Carnitine; Cholesterol; Cholesterol, HDL; Cholesterol, LDL; Female; Gestational Age; Humans; Infant, Low Birth Weight; Infant, Newborn; Korea; Male; Triglycerides

2007
A novel functional assay for simultaneous determination of total fatty acid beta-oxidation flux and acylcarnitine profiling in human skin fibroblasts using (2)H(31)-palmitate by isotope ratio mass spectrometry and electrospray tandem mass spectrometry.
    Clinica chimica acta; international journal of clinical chemistry, 2007, Volume: 382, Issue:1-2

    Topics: Carnitine; Cell Line; Deuterium; Fatty Acids; Fibroblasts; Humans; Lipid Metabolism, Inborn Errors; Mitochondria; Multienzyme Complexes; Oxidation-Reduction; Palmitates; Skin; Spectrometry, Mass, Electrospray Ionization; Tandem Mass Spectrometry

2007
Carnitine, acylcarnitine and amino acid profiles analyzed by tandem mass spectrometry in a surfactant/virus mouse model of acute hepatic encephalopathy.
    Chemosphere, 2007, Volume: 68, Issue:9

    Topics: Amino Acids; Animals; Carnitine; Disease Models, Animal; Hepatic Encephalopathy; Influenza B virus; Mice; Surface-Active Agents; Tandem Mass Spectrometry; Time Factors

2007
Prospective treatment in carnitine-acylcarnitine translocase deficiency.
    Journal of inherited metabolic disease, 2007, Volume: 30, Issue:5

    Topics: Carnitine; Carnitine Acyltransferases; Dicarboxylic Acids; Diet, Fat-Restricted; Dietary Supplements; Fatty Acids; Humans; Hyperammonemia; Hypoglycemia; Infant; Male; Membrane Transport Proteins; Metabolism, Inborn Errors; Oxidation-Reduction; Pedigree; Phenotype; Severity of Illness Index; Treatment Outcome

2007
ETFDH mutations as a major cause of riboflavin-responsive multiple acyl-CoA dehydrogenation deficiency.
    Brain : a journal of neurology, 2007, Volume: 130, Issue:Pt 8

    Topics: Acyl-CoA Dehydrogenase; Adolescent; Adult; Brain Diseases, Metabolic; Carnitine; Child; Child, Preschool; Electron Transport; Electron-Transferring Flavoproteins; Fatty Acids; Female; Humans; Iron-Sulfur Proteins; Male; Metabolism, Inborn Errors; Mitochondria, Muscle; Mitochondrial Myopathies; Muscle, Skeletal; Mutation; Oxidation-Reduction; Oxidoreductases Acting on CH-NH Group Donors; Riboflavin

2007
Expanded newborn screening in Europe 2007.
    Journal of inherited metabolic disease, 2007, Volume: 30, Issue:4

    Topics: Amino Acids; Carnitine; Europe; Humans; Infant, Newborn; Metabolism, Inborn Errors; Neonatal Screening; Outcome Assessment, Health Care; Spectrometry, Mass, Electrospray Ionization

2007
cis-3,4-Methylene-heptanoylcarnitine: characterization and verification of the C8:1 acylcarnitine in human urine.
    Journal of chromatography. B, Analytical technologies in the biomedical and life sciences, 2007, Oct-01, Volume: 857, Issue:2

    Topics: Carnitine; Chromatography, High Pressure Liquid; Esters; Gas Chromatography-Mass Spectrometry; Humans; Spectrometry, Mass, Electrospray Ionization

2007
Fatty acid chain-elongation in perfused rat heart: synthesis of stearoylcarnitine from perfused palmitate.
    FEBS letters, 2007, Sep-18, Volume: 581, Issue:23

    Topics: Animals; Carnitine; Chromatography, High Pressure Liquid; Fatty Acids; Glucose; Myocardium; Palmitates; Perfusion; Rats; Rats, Inbred F344

2007
A new LC-ESI-MS/MS method to measure long-chain acylcarnitine levels in cultured cells.
    Analytica chimica acta, 2007, Sep-05, Volume: 599, Issue:1

    Topics: Acetonitriles; Animals; Carnitine; Carnitine O-Palmitoyltransferase; Chromatography, High Pressure Liquid; Formates; Mitochondria; Palmitoyl Coenzyme A; PC12 Cells; Rats; Spectrometry, Mass, Electrospray Ionization; Tandem Mass Spectrometry; Transfection

2007
Choice of oils for essential fat supplements can enhance production of abnormal metabolites in fat oxidation disorders.
    Molecular genetics and metabolism, 2007, Volume: 92, Issue:4

    Topics: Acyl-CoA Dehydrogenase, Long-Chain; Carnitine; Cell Line; Diet Therapy; Dietary Fats; Energy Intake; Fibroblasts; Lipid Metabolism, Inborn Errors; Multienzyme Complexes; Oils; Oxidation-Reduction; Polyneuropathies

2007
Metabolomics identifies perturbations in human disorders of propionate metabolism.
    Clinical chemistry, 2007, Volume: 53, Issue:12

    Topics: Acetylcarnitine; Adult; Amino Acid Metabolism, Inborn Errors; Betaine; Biomarkers; Carnitine; Child; Chromatography, High Pressure Liquid; Humans; Methylmalonic Acid; Plasma; Propionates; Spectrometry, Mass, Electrospray Ionization

2007
Carnitine is associated with fatty acid metabolism in plants.
    Plant physiology and biochemistry : PPB, 2007, Volume: 45, Issue:12

    Topics: Arabidopsis; Brassica rapa; Carnitine; Chromatography, Gas; Chromatography, High Pressure Liquid; Fatty Acids; Flax; Nicotiana; Plants; Tandem Mass Spectrometry

2007
Separation and identification of plasma short-chain acylcarnitine isomers by HPLC/MS/MS for the differential diagnosis of fatty acid oxidation defects and organic acidemias.
    Journal of chromatography. B, Analytical technologies in the biomedical and life sciences, 2007, Dec-01, Volume: 860, Issue:1

    Topics: Carnitine; Chromatography, High Pressure Liquid; Female; Humans; Isomerism; Male; Tandem Mass Spectrometry

2007
No mutation was found in the alpha-subunit of the mitochondrial tri-functional protein in one patient with severe acute fatty liver of pregnancy and her relatives.
    Journal of gastroenterology and hepatology, 2007, Volume: 22, Issue:12

    Topics: 3-Hydroxyacyl CoA Dehydrogenases; Adult; Carnitine; Cytosine; Family; Fatty Liver; Female; Guanine; Humans; Long-Chain-3-Hydroxyacyl-CoA Dehydrogenase; Mass Spectrometry; Mitochondrial Trifunctional Protein; Multienzyme Complexes; Mutation; Polymorphism, Restriction Fragment Length; Pregnancy; Pregnancy Complications; Protein Subunits; Sequence Analysis, DNA

2007
[Application of tandem mass spectrometry on the diagnosis of fatty acid oxidation disorders].
    Zhonghua yi xue yi chuan xue za zhi = Zhonghua yixue yichuanxue zazhi = Chinese journal of medical genetics, 2007, Volume: 24, Issue:6

    Topics: Adolescent; Amino Acid Metabolism, Inborn Errors; Carnitine; Child; Child, Preschool; Female; Humans; Infant; Infant, Newborn; Lipid Metabolism; Lipid Metabolism, Inborn Errors; Male; Mass Spectrometry; Tandem Mass Spectrometry

2007
Acylcarnitine profiles during carnitine loading and fasting tests in a Japanese patient with medium-chain acyl-CoA dehydrogenase deficiency.
    The Tohoku journal of experimental medicine, 2007, Volume: 213, Issue:4

    Topics: Acyl-CoA Dehydrogenase; Asian People; Blood Glucose; Carnitine; Child; Child, Preschool; Diagnostic Tests, Routine; DNA Mutational Analysis; Fasting; Female; Humans; Japan; Lipid Metabolism, Inborn Errors

2007
A new case of short-chain acyl-CoA dehydrogenase deficiency: clinical, biochemical, genetic and (1)H-NMR spectroscopic studies.
    Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology, 2007, Volume: 28, Issue:6

    Topics: Adult; Butyryl-CoA Dehydrogenase; Carnitine; Chromatography, Gas; Humans; Magnetic Resonance Spectroscopy; Male; Metabolism, Inborn Errors

2007
Interaction of mildronate with the mitochondrial carnitine/acylcarnitine transport protein.
    Journal of biochemical and molecular toxicology, 2008, Volume: 22, Issue:1

    Topics: Animals; Antiporters; Biological Transport; Carnitine; Dose-Response Relationship, Drug; Kinetics; Liposomes; Membrane Transport Proteins; Methylhydrazines; Mitochondria, Liver; Protein Binding; Rats; Time Factors

2008
Metabolic testing in the pediatric epilepsy unit.
    Pediatric neurology, 2008, Volume: 38, Issue:3

    Topics: Adolescent; Adult; Amino Acids; Ammonia; Carnitine; Child; Child, Preschool; Electroencephalography; Epilepsy; Female; Follow-Up Studies; Humans; Infant; Magnetic Resonance Imaging; Male; Pediatrics; Retrospective Studies

2008
Combined newborn screening for succinylacetone, amino acids, and acylcarnitines in dried blood spots.
    Clinical chemistry, 2008, Volume: 54, Issue:4

    Topics: Amino Acids; Blood Specimen Collection; Carnitine; Flow Injection Analysis; Heptanoates; Humans; Infant, Newborn; Neonatal Screening; Retrospective Studies; Tandem Mass Spectrometry; Tyrosinemias

2008
Acylcarnitine profile analysis.
    Genetics in medicine : official journal of the American College of Medical Genetics, 2008, Volume: 10, Issue:2

    Topics: Biomarkers; Carnitine; Genes, Recessive; Genetics, Medical; Humans; Metabolism, Inborn Errors; Prevalence; Spectrometry, Mass, Electrospray Ionization; Tandem Mass Spectrometry

2008
Carnitine supplementation induces acylcarnitine production in tissues of very long-chain acyl-CoA dehydrogenase-deficient mice, without replenishing low free carnitine.
    Pediatric research, 2008, Volume: 63, Issue:6

    Topics: Acyl-CoA Dehydrogenase, Long-Chain; Animals; Betaine; Carnitine; Cell Line, Tumor; Cell Proliferation; Cell Survival; Dietary Supplements; Humans; Lipid Metabolism, Inborn Errors; Liver; Mice; Mice, Knockout; Muscle, Skeletal; Palmitoyl Coenzyme A; Palmitoylcarnitine; Physical Exertion; Time Factors

2008
Enhanced acyl-CoA dehydrogenase activity is associated with improved mitochondrial and contractile function in heart failure.
    Cardiovascular research, 2008, Jul-15, Volume: 79, Issue:2

    Topics: Acyl-CoA Dehydrogenase; Adiponectin; Animals; Blood Glucose; Carnitine; Dietary Fats; Disease Models, Animal; Electron Transport Chain Complex Proteins; Fatty Acids, Nonesterified; Heart Failure; Insulin; Leptin; Male; Mitochondria, Heart; Myocardial Contraction; Rats; Rats, Wistar; Triglycerides

2008
Bioinformatics analysis of targeted metabolomics--uncovering old and new tales of diabetic mice under medication.
    Endocrinology, 2008, Volume: 149, Issue:7

    Topics: Amino Acids; Animals; Carnitine; Computational Biology; Diabetes Mellitus; Gluconeogenesis; Hypoglycemic Agents; Male; Mice; Mice, Inbred C57BL; Models, Biological; Rosiglitazone; Spectrometry, Mass, Electrospray Ionization; Tandem Mass Spectrometry; Thiazolidinediones

2008
Enhancing newborn screening for tyrosinemia type I.
    Clinical chemistry, 2008, Volume: 54, Issue:4

    Topics: Amino Acids; Carnitine; Heptanoates; Humans; Infant, Newborn; Neonatal Screening; Tyrosinemias

2008
Chronic suppression of acetyl-CoA carboxylase 1 in beta-cells impairs insulin secretion via inhibition of glucose rather than lipid metabolism.
    The Journal of biological chemistry, 2008, May-23, Volume: 283, Issue:21

    Topics: Acetyl-CoA Carboxylase; Adenosine Diphosphate; Adenosine Triphosphate; Animals; Carnitine; Cell Line; Fatty Acids; Gene Expression Regulation, Enzymologic; Glucose; Insulin; Insulin Secretion; Insulin-Secreting Cells; Isoenzymes; Lipid Metabolism; Oxidation-Reduction; Pyruvic Acid; Rats; RNA, Small Interfering; Time Factors

2008
Acquired multiple Acyl-CoA dehydrogenase deficiency in 10 horses with atypical myopathy.
    Neuromuscular disorders : NMD, 2008, Volume: 18, Issue:5

    Topics: Acyl-CoA Dehydrogenase; Acyl-CoA Dehydrogenases; Animals; Butyric Acid; Butyryl-CoA Dehydrogenase; Carnitine; Chromatography, High Pressure Liquid; Female; Gas Chromatography-Mass Spectrometry; Glutarates; Horse Diseases; Horses; Isovaleryl-CoA Dehydrogenase; Lactic Acid; Male; Microscopy, Electron; Microscopy, Fluorescence; Muscles; Muscular Diseases; Riboflavin

2008
Impact of short- and medium-chain organic acids, acylcarnitines, and acyl-CoAs on mitochondrial energy metabolism.
    Biochimica et biophysica acta, 2008, Volume: 1777, Issue:10

    Topics: Acids; Acyl Coenzyme A; Animals; Carnitine; Cattle; Electron Transport Complex III; Energy Metabolism; Humans; Mitochondria; Reye Syndrome

2008
[New therapeutic approaches in mitochondrial fatty acid oxidation disorders].
    Archives de pediatrie : organe officiel de la Societe francaise de pediatrie, 2008, Volume: 15, Issue:5

    Topics: Carnitine; Citric Acid Cycle; Fatty Acids; Humans; Mitochondrial Diseases; Mitochondrial Trifunctional Protein; Multienzyme Complexes

2008
Identification of new mutations in the ETHE1 gene in a cohort of 14 patients presenting with ethylmalonic encephalopathy.
    Journal of medical genetics, 2008, Volume: 45, Issue:7

    Topics: Adolescent; Amino Acid Sequence; Base Sequence; Brain Diseases, Metabolic, Inborn; Carnitine; Child; Child, Preschool; Cohort Studies; DNA; Female; Humans; Infant; Male; Mitochondrial Proteins; Models, Molecular; Molecular Sequence Data; Mutation, Missense; Nucleocytoplasmic Transport Proteins; Polymerase Chain Reaction; Polymorphism, Single Nucleotide; Sequence Alignment

2008
Application of metabolomic principles to disorders of nucleotide metabolism reveals new metabolic perturbations.
    Nucleosides, nucleotides & nucleic acids, 2008, Volume: 27, Issue:6

    Topics: Adenine Phosphoribosyltransferase; Adenosine Deaminase; Amino Acids; Carnitine; Dihydropyrimidine Dehydrogenase Deficiency; Dihydrouracil Dehydrogenase (NADP); Genetic Diseases, Inborn; Humans; Hypoxanthine Phosphoribosyltransferase; Metabolic Diseases; Ninhydrin; Nucleotides

2008
[Neonatal onset of organic acidemia (propionic) diagnosed by tandem mass spectrometry].
    Biomedica : revista del Instituto Nacional de Salud, 2008, Volume: 28, Issue:1

    Topics: Amino Acid Metabolism, Inborn Errors; Carnitine; Consanguinity; Fatal Outcome; Female; Humans; Infant, Newborn; Propionates; Tandem Mass Spectrometry

2008
Determination of the reference range of endogenous plasma carnitines in healthy adults.
    Annals of clinical biochemistry, 2008, Volume: 45, Issue:Pt 6

    Topics: Acetylcarnitine; Adolescent; Adult; Age Factors; Blood Chemical Analysis; Carnitine; Chromatography, High Pressure Liquid; Female; Humans; Male; Reference Values; Sex Characteristics; Spectrometry, Mass, Electrospray Ionization; Young Adult

2008
Urine acylcarnitine analysis by ESI-MS/MS: a new tool for the diagnosis of peroxisomal biogenesis disorders.
    Clinica chimica acta; international journal of clinical chemistry, 2008, Volume: 398, Issue:1-2

    Topics: Carnitine; Female; Humans; Infant; Infant, Newborn; Male; Peroxisomal Disorders; Refsum Disease; Reproducibility of Results; Spectrometry, Mass, Electrospray Ionization; Tandem Mass Spectrometry; Zellweger Syndrome

2008
Liquid chromatography/mass spectrometry determination of endogenous plasma acetyl and palmitoyl carnitines as potential biomarkers of beta-oxidation in mice.
    Rapid communications in mass spectrometry : RCM, 2008, Volume: 22, Issue:21

    Topics: Animals; Biomarkers; Calibration; Carnitine; Chromatography, Liquid; Isotope Labeling; Male; Mass Spectrometry; Mice; Mice, Inbred Strains; Molecular Structure; Oxidation-Reduction; Palmitoylcarnitine; Reference Standards; Reproducibility of Results; Sensitivity and Specificity

2008
Carnitine-palmitoyltransferase 2 deficiency: novel mutations and relevance of newborn screening.
    American journal of medical genetics. Part A, 2008, Nov-15, Volume: 146A, Issue:22

    Topics: Carnitine; Carnitine O-Palmitoyltransferase; Child, Preschool; Humans; Infant, Newborn; Male; Mutation; Mutation, Missense; Neonatal Screening; Phenotype; Sequence Deletion

2008
Muscle acylcarnitines during short-term fasting in lean healthy men.
    Clinical science (London, England : 1979), 2009, Volume: 116, Issue:7

    Topics: Adult; Anthropometry; Blood Glucose; Calorimetry, Indirect; Carnitine; Fasting; Humans; Insulin Resistance; Lipid Metabolism; Male; Muscle, Skeletal; Oxidation-Reduction; Oxygen Consumption; Young Adult

2009
Precursor ion scan profiles of acylcarnitines by atmospheric pressure thermal desorption chemical ionization tandem mass spectrometry.
    Rapid communications in mass spectrometry : RCM, 2008, Volume: 22, Issue:23

    Topics: Atmospheric Pressure; Carnitine; Humans; Spectrometry, Mass, Electrospray Ionization; Tandem Mass Spectrometry; Temperature

2008
Plasma acylcarnitine and fatty acid profiles during exercise and training in Standardbreds.
    American journal of veterinary research, 2008, Volume: 69, Issue:11

    Topics: 3-Hydroxybutyric Acid; Analysis of Variance; Animals; Carnitine; Fatty Acids; Gas Chromatography-Mass Spectrometry; Horses; Lactic Acid; Male; Muscle, Skeletal; Physical Conditioning, Animal

2008
Corresponding increase in long-chain acyl-CoA and acylcarnitine after exercise in muscle from VLCAD mice.
    Journal of lipid research, 2009, Volume: 50, Issue:8

    Topics: Acyl Coenzyme A; Acyl-CoA Dehydrogenase, Long-Chain; Animals; Body Weight; Carnitine; Mice; Mice, Inbred C57BL; Mice, Knockout; Mitochondria, Muscle; Muscle, Skeletal; Physical Exertion

2009
Proteomic changes associated with diabetes in the BB-DP rat.
    American journal of physiology. Endocrinology and metabolism, 2009, Volume: 296, Issue:3

    Topics: Animals; Carnitine; Citrate (si)-Synthase; Diabetes Mellitus, Type 1; Disease Models, Animal; Electrophoresis, Gel, Two-Dimensional; Gluconeogenesis; Glycolysis; Liver; Male; Methionine; Muscle, Skeletal; Myocardium; Proteomics; Rats; Rats, Inbred BB; Reactive Oxygen Species

2009
Challenges and developments in tandem mass spectrometry based clinical metabolomics.
    Molecular and cellular endocrinology, 2009, Mar-25, Volume: 301, Issue:1-2

    Topics: Amino Acids; Biometry; Carnitine; Eicosanoids; Estradiol; Gas Chromatography-Mass Spectrometry; Humans; Infant, Newborn; Macrophages; Metabolome; Metabolomics; Neonatal Screening; Tandem Mass Spectrometry; Testosterone

2009
Changes of the plasma metabolome during an oral glucose tolerance test: is there more than glucose to look at?
    American journal of physiology. Endocrinology and metabolism, 2009, Volume: 296, Issue:2

    Topics: Bile Acids and Salts; Blood; Blood Chemical Analysis; Blood Glucose; Blood Proteins; Carnitine; Fatty Acids, Nonesterified; Glucose; Glucose Tolerance Test; Humans; Insulin; Lysophosphatidylcholines; Metabolome; Middle Aged

2009
Newborn screening of inborn errors of metabolism by capillary electrophoresis-electrospray ionization-mass spectrometry: a second-tier method with improved specificity and sensitivity.
    Analytical chemistry, 2009, Jan-01, Volume: 81, Issue:1

    Topics: Adult; Amino Acids; Calibration; Carnitine; Electrophoresis, Capillary; Humans; Infant, Newborn; Mass Screening; Metabolism, Inborn Errors; Reproducibility of Results; Sensitivity and Specificity; Spectrometry, Mass, Electrospray Ionization

2009
Bloodspot acylcarnitine and amino acid analysis in cord blood samples: efficacy and reference data from a large cohort study.
    Journal of inherited metabolic disease, 2009, Volume: 32, Issue:1

    Topics: Amino Acids; Blood Chemical Analysis; Blood Specimen Collection; Carnitine; Cohort Studies; Efficiency; False Negative Reactions; Fetal Blood; Fetal Diseases; Humans; Infant, Newborn; Metabolic Diseases; Mothers; Neonatal Screening; Reference Values; Time Factors

2009
Atypical presentation of VLCAD deficiency associated with a novel ACADVL splicing mutation.
    Muscle & nerve, 2009, Volume: 39, Issue:3

    Topics: Acyl-CoA Dehydrogenase, Long-Chain; Calcium Channel Blockers; Carnitine; DNA Mutational Analysis; Female; Humans; Lamotrigine; Lipid Metabolism, Inborn Errors; Muscle, Skeletal; Mutation; Succinate Dehydrogenase; Triazines; Young Adult

2009
Serum metabolomics reveals irreversible inhibition of fatty acid beta-oxidation through the suppression of PPARalpha activation as a contributing mechanism of acetaminophen-induced hepatotoxicity.
    Chemical research in toxicology, 2009, Volume: 22, Issue:4

    Topics: Acetaminophen; Animals; Carnitine; Chemical and Drug Induced Liver Injury; Chromatography, Liquid; Cytochrome P-450 CYP2E1; Fatty Acids; Glutathione; Liver Diseases; Mass Spectrometry; Metabolomics; Mice; Mice, Knockout; PPAR alpha; Time Factors

2009
Site-directed mutagenesis of the His residues of the rat mitochondrial carnitine/acylcarnitine carrier: implications for the role of His-29 in the transport pathway.
    Biochimica et biophysica acta, 2009, Volume: 1787, Issue:8

    Topics: Amino Acid Sequence; Animals; Biological Transport; Carnitine; Carnitine Acyltransferases; Carrier Proteins; Escherichia coli; Histidine; Hydrogen-Ion Concentration; Kinetics; Mitochondrial Proteins; Models, Molecular; Molecular Sequence Data; Mutagenesis, Site-Directed; Rats; Recombinant Proteins; Sequence Alignment

2009
Metabolomic profiling reveals distinct patterns of myocardial substrate use in humans with coronary artery disease or left ventricular dysfunction during surgical ischemia/reperfusion.
    Circulation, 2009, Apr-07, Volume: 119, Issue:13

    Topics: Aged; Anaerobic Threshold; Cardiac Output; Cardiopulmonary Bypass; Carnitine; Coronary Artery Disease; Female; Heart; Heart Arrest, Induced; Heart Diseases; Humans; Intraoperative Complications; Lactic Acid; Male; Metabolome; Middle Aged; Myocardial Reperfusion Injury; Myocardium; Postoperative Complications; Ventricular Dysfunction, Left

2009
Stability of acylcarnitines and free carnitine in dried blood samples: implications for retrospective diagnosis of inborn errors of metabolism and neonatal screening for carnitine transporter deficiency.
    Analytical chemistry, 2009, May-01, Volume: 81, Issue:9

    Topics: Carnitine; Desiccation; Humans; Infant, Newborn; Linear Models; Metabolism, Inborn Errors; Neonatal Screening; Organic Cation Transport Proteins; Reproducibility of Results; Retrospective Studies; Solute Carrier Family 22 Member 5; Specimen Handling; Spectrometry, Mass, Electrospray Ionization; Tandem Mass Spectrometry; Temperature

2009
Diagnostic assessment and long-term follow-up of 13 patients with Very Long-Chain Acyl-Coenzyme A dehydrogenase (VLCAD) deficiency.
    Neuromuscular disorders : NMD, 2009, Volume: 19, Issue:5

    Topics: Acyl-CoA Dehydrogenase, Long-Chain; Adolescent; Adult; Biomarkers; Carnitine; Cells, Cultured; Child; DNA Mutational Analysis; Exercise Tolerance; Female; Genetic Testing; Genotype; Heterozygote; Homozygote; Humans; Male; Metabolism, Inborn Errors; Middle Aged; Mitochondrial Diseases; Muscle Weakness; Muscular Diseases; Mutation; Rhabdomyolysis; Young Adult

2009
Addition of formic acid improves acetonitrile extraction of dicarboxylic acylcarnitines.
    Clinica chimica acta; international journal of clinical chemistry, 2009, Jun-27, Volume: 404, Issue:2

    Topics: Acetonitriles; Blood Chemical Analysis; Carnitine; Formates; Humans; Metabolic Diseases; Solvents

2009
High heritability of metabolomic profiles in families burdened with premature cardiovascular disease.
    Molecular systems biology, 2009, Volume: 5

    Topics: Age of Onset; Amino Acids; Cardiovascular Diseases; Carnitine; Family Health; Fatty Acids; Female; Humans; Inheritance Patterns; Male; Metabolome; Middle Aged; Principal Component Analysis

2009
Plasma acylcarnitine profiles suggest incomplete long-chain fatty acid beta-oxidation and altered tricarboxylic acid cycle activity in type 2 diabetic African-American women.
    The Journal of nutrition, 2009, Volume: 139, Issue:6

    Topics: Adult; Aged; Aged, 80 and over; Black or African American; Carnitine; Citric Acid Cycle; Diabetes Mellitus, Type 2; Fatty Acids; Female; Humans; Ion Channels; Middle Aged; Mitochondrial Proteins; Mutation, Missense; NF-kappa B; Obesity; Oxidation-Reduction; Polymorphism, Genetic; Uncoupling Protein 3; Young Adult

2009
Deuterated palmitate-driven acylcarnitine formation by whole-blood samples for a rapid diagnostic exploration of mitochondrial fatty acid oxidation disorders.
    Clinica chimica acta; international journal of clinical chemistry, 2009, Volume: 406, Issue:1-2

    Topics: Acyl-CoA Dehydrogenases; Adult; Blood Specimen Collection; Carnitine; Case-Control Studies; Child; Child, Preschool; Deuterium; Fatty Acids; Female; Humans; Infant; Infant, Newborn; Kinetics; Male; Mitochondrial Diseases; Mutation; Oxidation-Reduction; Palmitates; Time Factors

2009
Evidence for the participation of the stimulated sympathetic nervous system in the regulation of carnitine blood levels of soccer players during a game.
    Metabolism: clinical and experimental, 2009, Volume: 58, Issue:8

    Topics: Adolescent; Antioxidants; Biomarkers; Carnitine; Chromatography, High Pressure Liquid; Creatine Kinase; Epinephrine; Humans; L-Lactate Dehydrogenase; Lactic Acid; Male; Norepinephrine; Pyruvic Acid; Soccer; Sympathetic Nervous System; Tandem Mass Spectrometry; Time Factors; Young Adult

2009
Mitochondrial long chain fatty acid beta-oxidation in man and mouse.
    Biochimica et biophysica acta, 2009, Volume: 1791, Issue:8

    Topics: Acyl-CoA Dehydrogenase, Long-Chain; Animals; Carnitine; Cell Line; Chromatography, High Pressure Liquid; Fatty Acids; Fibroblasts; Humans; Mice; Mitochondria; Oxidation-Reduction; Tandem Mass Spectrometry

2009
Relationships between circulating metabolic intermediates and insulin action in overweight to obese, inactive men and women.
    Diabetes care, 2009, Volume: 32, Issue:9

    Topics: Adolescent; Adult; Aged; Amino Acids; Blood Glucose; Carnitine; Cross-Sectional Studies; Fatty Acids, Nonesterified; Female; Gas Chromatography-Mass Spectrometry; Humans; Insulin Resistance; Male; Middle Aged; Obesity; Overweight; Principal Component Analysis; Tandem Mass Spectrometry; Young Adult

2009
Improved MS/MS analysis of succinylacetone extracted from dried blood spots when combined with amino acids and acylcarnitine butyl esters.
    Clinica chimica acta; international journal of clinical chemistry, 2009, Volume: 407, Issue:1-2

    Topics: Amino Acids; Artifacts; Blood Chemical Analysis; Carnitine; Esters; Heptanoates; Humans; Hydrazines; Methanol; Specimen Handling; Tandem Mass Spectrometry

2009
Carnitine insufficiency caused by aging and overnutrition compromises mitochondrial performance and metabolic control.
    The Journal of biological chemistry, 2009, Aug-21, Volume: 284, Issue:34

    Topics: Aging; Animals; Biological Transport; Blotting, Western; Carnitine; Carnitine O-Acetyltransferase; Cells, Cultured; Dietary Fats; gamma-Butyrobetaine Dioxygenase; Glucose Intolerance; Glucose Tolerance Test; Humans; Lipid Metabolism; Male; Mitochondria, Muscle; Mixed Function Oxygenases; Overnutrition; Oxidative Phosphorylation; Random Allocation; Rats; Rats, Wistar; Reverse Transcriptase Polymerase Chain Reaction; Vitamin B Complex

2009
Effect of heat stress and bezafibrate on mitochondrial beta-oxidation: comparison between cultured cells from normal and mitochondrial fatty acid oxidation disorder children using in vitro probe acylcarnitine profiling assay.
    Brain & development, 2010, Volume: 32, Issue:5

    Topics: Acyl-CoA Dehydrogenase; Acyl-CoA Dehydrogenase, Long-Chain; Bezafibrate; Carnitine; Cells, Cultured; Child; Fatty Acids; Fibroblasts; Hot Temperature; Humans; Hypolipidemic Agents; Lipid Metabolism, Inborn Errors; Mitochondria; Mitochondrial Diseases; Oxidation-Reduction; Stress, Physiological

2010
[Multiple acyl-CoA dehydrogenase deficiency (MADD): a curable cause of genetic muscular lipidosis].
    Revue neurologique, 2010, Volume: 166, Issue:3

    Topics: Acyl-CoA Dehydrogenases; Adult; Biopsy; Brain Diseases, Metabolic; Carnitine; Coloring Agents; DNA Mutational Analysis; Electron Transport; Electron-Transferring Flavoproteins; Female; Humans; Lipid Metabolism, Inborn Errors; Lipidoses; Male; Middle Aged; Muscle Weakness; Muscle, Skeletal; Riboflavin; Tandem Mass Spectrometry; Young Adult

2010
Statins, fibrates and retinoic acid upregulate mitochondrial acylcarnitine carrier gene expression.
    Biochemical and biophysical research communications, 2009, Oct-30, Volume: 388, Issue:4

    Topics: Alitretinoin; Animals; Anticholesteremic Agents; Carnitine; Clofibric Acid; Colforsin; Gene Expression; Humans; Hydroxymethylglutaryl-CoA Reductase Inhibitors; Membrane Transport Proteins; Mitochondria; PPAR alpha; Promoter Regions, Genetic; Rats; Tretinoin

2009
A neonatal death due to medium-chain acyl-CoA dehydrogenase deficiency: utilization of the neonatal metabolic screen in a functional approach to sudden unexplained infant death.
    The American journal of forensic medicine and pathology, 2009, Volume: 30, Issue:3

    Topics: Acyl-CoA Dehydrogenase; Carnitine; Gene Deletion; Humans; Infant, Newborn; Lipid Metabolism, Inborn Errors; Male; Neonatal Screening; Point Mutation; Sudden Infant Death

2009
Searching for the needle in the Haystacks.
    Lancet (London, England), 2009, Sep-05, Volume: 374, Issue:9692

    Topics: Acyl-CoA Dehydrogenase, Long-Chain; Age of Onset; Biopsy; Carnitine; Creatine Kinase; Creatinine; Histocytochemistry; Humans; Male; Middle Aged; Mitochondrial Myopathies; Prognosis; Rare Diseases; Recurrence; Rhabdomyolysis; Walking

2009
Dissection of biochemical borderline phenotypes in carriers and genetic variants of medium-chain acyl-CoA dehyrogenase deficiency: implications for newborn screening [corrected].
    Clinical genetics, 2009, Volume: 76, Issue:2

    Topics: Carnitine; Case-Control Studies; Heterozygote; Humans; Infant, Newborn; Lipid Metabolism, Inborn Errors; Mutation; Neonatal Screening

2009
Variation in the human lipidome associated with coffee consumption as revealed by quantitative targeted metabolomics.
    Molecular nutrition & food research, 2009, Volume: 53, Issue:11

    Topics: Aged; Atherosclerosis; Carnitine; Cholesterol; Coffee; Humans; Male; Metabolomics; Middle Aged; Sphingomyelins; Tandem Mass Spectrometry

2009
Identification of a neonate with hepatorenal tyrosinemia by combined routine newborn screening for succinylacetone, acylcarnitines and amino acids.
    Clinical biochemistry, 2010, Volume: 43, Issue:7-8

    Topics: Amino Acids; Carnitine; Heptanoates; Humans; Infant, Newborn; Kidney Diseases; Liver Diseases; Neonatal Screening; Tandem Mass Spectrometry; Tyrosinemias

2010
Newborn screening and early biochemical follow-up in combined methylmalonic aciduria and homocystinuria, cblC type, and utility of methionine as a secondary screening analyte.
    Molecular genetics and metabolism, 2010, Volume: 99, Issue:2

    Topics: Algorithms; Carnitine; Demography; False Positive Reactions; Female; Follow-Up Studies; Genetic Association Studies; Homocystinuria; Humans; Infant, Newborn; Male; Metabolism, Inborn Errors; Methionine; Methylmalonic Acid; Neonatal Screening; New York; Propionates; Referral and Consultation; Vitamin B 12

2010
Relationship between plasma free fatty acid, intramyocellular triglycerides and long-chain acylcarnitines in resting humans.
    The Journal of physiology, 2009, Dec-15, Volume: 587, Issue:Pt 24

    Topics: Adult; Carnitine; Fatty Acids, Nonesterified; Female; Humans; Male; Muscle Fibers, Skeletal; Rest; Triglycerides

2009
Two novel HADHB gene mutations in a Korean patient with mitochondrial trifunctional protein deficiency.
    Annals of clinical and laboratory science, 2009,Fall, Volume: 39, Issue:4

    Topics: 3-Hydroxyacyl CoA Dehydrogenases; Cardiomyopathies; Carnitine; DNA Mutational Analysis; Humans; Infant, Newborn; Lipid Metabolism, Inborn Errors; Male; Mitochondria; Mitochondrial Myopathies; Mitochondrial Proteins; Mitochondrial Trifunctional Protein; Mitochondrial Trifunctional Protein, beta Subunit; Multienzyme Complexes; Mutation; Nervous System Diseases; Peripheral Nervous System Diseases; Republic of Korea; Retinitis Pigmentosa; Rhabdomyolysis

2009
Mechanisms underlying metabolic and neural defects in zebrafish and human multiple acyl-CoA dehydrogenase deficiency (MADD).
    PloS one, 2009, Dec-17, Volume: 4, Issue:12

    Topics: Animals; Carboxylic Acids; Carnitine; Cell Proliferation; Cloning, Molecular; Electron-Transferring Flavoproteins; Enzyme Activation; Extracellular Signal-Regulated MAP Kinases; Fibroblasts; Glycolysis; Humans; Infant; Infant, Newborn; Iron-Sulfur Proteins; Mitochondria; Multiple Acyl Coenzyme A Dehydrogenase Deficiency; Mutation; Nervous System; Neurons; Oligonucleotides, Antisense; Oxidoreductases Acting on CH-NH Group Donors; Peroxisome Proliferator-Activated Receptors; Phenotype; Zebrafish

2009
Diagnosis, treatment, and long-term outcomes of late-onset (type III) multiple acyl-CoA dehydrogenase deficiency.
    Journal of child neurology, 2010, Volume: 25, Issue:8

    Topics: Brain Diseases, Metabolic; Carnitine; Child; Child, Preschool; Developmental Disabilities; Female; Humans; Male; Mass Screening; Metabolic Diseases; Multiple Acyl Coenzyme A Dehydrogenase Deficiency

2010
Increased mitochondrial content rescues in vivo muscle oxidative capacity in long-term high-fat-diet-fed rats.
    FASEB journal : official publication of the Federation of American Societies for Experimental Biology, 2010, Volume: 24, Issue:5

    Topics: Animals; Carnitine; Diet; Dietary Fats; Insulin Resistance; Male; Mitochondria, Muscle; Muscle, Skeletal; Oxidation-Reduction; Oxygen Consumption; Rats; Rats, Wistar

2010
Study of inborn errors of metabolism in urine from patients with unexplained mental retardation.
    Journal of inherited metabolic disease, 2010, Volume: 33, Issue:1

    Topics: Adenylosuccinate Lyase; Adolescent; Adult; Aged; Carnitine; Child; Child, Preschool; Chromium; Cohort Studies; Creatine; Female; Humans; Intellectual Disability; Male; Metabolism, Inborn Errors; Middle Aged; Phenylketonurias; Purines; Pyrimidines

2010
Acylcarnitine analysis by tandem mass spectrometry.
    Current protocols in human genetics, 2010, Volume: Chapter 17

    Topics: Carnitine; Flow Injection Analysis; Humans; Lipid Metabolism, Inborn Errors; Spectrometry, Mass, Electrospray Ionization; Tandem Mass Spectrometry

2010
Preeclampsia and HELLP syndrome: impaired mitochondrial function in umbilical endothelial cells.
    Reproductive sciences (Thousand Oaks, Calif.), 2010, Volume: 17, Issue:3

    Topics: Adult; Carnitine; Carnitine O-Palmitoyltransferase; Citrate (si)-Synthase; Electron Transport; Endothelial Cells; Energy Metabolism; Fatty Acids; Female; Glycolysis; HELLP Syndrome; Humans; Infant, Newborn; L-Lactate Dehydrogenase; Mitochondria; Mitochondrial Diseases; Multienzyme Complexes; Oxidation-Reduction; Phosphofructokinases; Pre-Eclampsia; Pregnancy; Umbilical Veins

2010
Cardiac and plasma lipid profiles in response to acute hypoxia in neonatal and young adult rats.
    Lipids in health and disease, 2010, Jan-13, Volume: 9

    Topics: Animals; Carnitine; Dietary Fats; Fatty Acids; Female; Hypoxia; Lipids; Male; Models, Biological; Myocardium; Rats; Rats, Sprague-Dawley; Time Factors

2010
Measurement of plasma/serum acylcarnitines using tandem mass spectrometry.
    Methods in molecular biology (Clifton, N.J.), 2010, Volume: 603

    Topics: Carnitine; Humans; Lipid Metabolism, Inborn Errors; Pediatrics; Spectrometry, Mass, Electrospray Ionization; Tandem Mass Spectrometry

2010
Comparison of amino acids and acylcarnitines assay methods used in newborn screening assays by tandem mass spectrometry.
    Clinica chimica acta; international journal of clinical chemistry, 2010, May-02, Volume: 411, Issue:9-10

    Topics: Amino Acids; Butanols; Carnitine; Humans; Infant, Newborn; Leucine; Metabolic Diseases; Methionine; Neonatal Screening; Palmitoylcarnitine; Phenylalanine; Quality Control; Tandem Mass Spectrometry

2010
LMNA mutations, skeletal muscle lipid metabolism, and insulin resistance.
    The Journal of clinical endocrinology and metabolism, 2010, Volume: 95, Issue:4

    Topics: Adult; Blood Glucose; Carnitine; Cells, Cultured; Energy Metabolism; Female; Glycogen; Humans; Insulin; Insulin Resistance; Lamin Type A; Lipid Metabolism; Lipodystrophy, Familial Partial; Male; Middle Aged; Muscle Fibers, Skeletal; Muscle, Skeletal; Muscular Dystrophies, Limb-Girdle; Mutation; Oligonucleotide Array Sequence Analysis; Oxidation-Reduction; Phenotype

2010
The first case of mitochondrial acetoacetyl-CoA thiolase deficiency identified by expanded newborn metabolic screening in Italy: the importance of an integrated diagnostic approach.
    Journal of inherited metabolic disease, 2010, Volume: 33 Suppl 3

    Topics: Acetyl-CoA C-Acetyltransferase; Acetyl-CoA C-Acyltransferase; Amino Acid Metabolism, Inborn Errors; Biomarkers; Carnitine; Chromatography, Liquid; DNA Mutational Analysis; Dried Blood Spot Testing; Genetic Predisposition to Disease; Genetic Testing; Heredity; Humans; Infant, Newborn; Italy; Mutation; Neonatal Screening; Pedigree; Phenotype; Predictive Value of Tests; Tandem Mass Spectrometry

2010
Serum carnitine levels in childhood leukemia.
    Journal of pediatric hematology/oncology, 2010, Volume: 32, Issue:2

    Topics: Acute Disease; Adolescent; Body Mass Index; Carnitine; Child; Child, Preschool; Female; Humans; Leukemia; Male; Muscle, Skeletal

2010
Four years of expanded newborn screening in Portugal with tandem mass spectrometry.
    Journal of inherited metabolic disease, 2010, Volume: 33 Suppl 3

    Topics: Amino Acids; Biomarkers; Carnitine; Early Diagnosis; False Positive Reactions; Genetic Predisposition to Disease; Humans; Infant, Newborn; Metabolism, Inborn Errors; Neonatal Screening; Phenotype; Portugal; Predictive Value of Tests; Prevalence; Prognosis; Program Evaluation; Tandem Mass Spectrometry; Time Factors

2010
SIRT3 regulates mitochondrial fatty-acid oxidation by reversible enzyme deacetylation.
    Nature, 2010, Mar-04, Volume: 464, Issue:7285

    Topics: Acetylation; Acyl-CoA Dehydrogenase, Long-Chain; Adenosine Triphosphate; Adipose Tissue, Brown; Animals; Body Temperature Regulation; Caloric Restriction; Carnitine; Cell Line; Cold Temperature; Fasting; Fatty Acids; Humans; Hypoglycemia; Liver; Male; Mass Spectrometry; Mice; Mitochondria; Oxidation-Reduction; Sirtuin 3; Triglycerides; Up-Regulation

2010
Heat stress deteriorates mitochondrial beta-oxidation of long-chain fatty acids in cultured fibroblasts with fatty acid beta-oxidation disorders.
    Journal of chromatography. B, Analytical technologies in the biomedical and life sciences, 2010, Jun-15, Volume: 878, Issue:20

    Topics: Adolescent; Carnitine; Child; Fatty Acids; Female; Fibroblasts; Heat Stress Disorders; Humans; Lipid Metabolism, Inborn Errors; Male; Mitochondria; Oxidation-Reduction; Tandem Mass Spectrometry

2010
Metabolomic analyses of plasma reveals new insights into asphyxia and resuscitation in pigs.
    PloS one, 2010, Mar-09, Volume: 5, Issue:3

    Topics: Animals; Animals, Newborn; Asphyxia; Blood Proteins; Carnitine; Female; Glycine; Hydrogen-Ion Concentration; Hypoxia; Lactates; Male; Metabolomics; Oxygen; Resuscitation; Swine

2010
Hypoketotic hypoglycemia with myolysis and hypoparathyroidism: an unusual association in medium chain acyl-CoA desydrogenase deficiency (MCADD).
    Journal of pediatric endocrinology & metabolism : JPEM, 2009, Volume: 22, Issue:12

    Topics: Acute Kidney Injury; Acyl-CoA Dehydrogenase; Carnitine; Child, Preschool; Female; Glucose; Humans; Hypoglycemia; Hypoparathyroidism; Ketones; Lipid Metabolism, Inborn Errors; Mutation, Missense; Vitamin B Complex

2009
Elevated hydroxyacylcarnitines in a carrier of LCHAD deficiency during acute liver disease of pregnancy - a common feature of the pregnancy complication?
    Molecular genetics and metabolism, 2010, Volume: 100, Issue:2

    Topics: 3-Hydroxyacyl CoA Dehydrogenases; Acute Disease; Adult; Carnitine; Fatty Liver; Female; Humans; Lipid Metabolism, Inborn Errors; Liver Diseases; Long-Chain-3-Hydroxyacyl-CoA Dehydrogenase; Mitochondrial Trifunctional Protein; Multienzyme Complexes; Pregnancy; Pregnancy Complications; Pregnancy Trimester, Third

2010
In vitro probe acylcarnitine profiling assay using cultured fibroblasts and electrospray ionization tandem mass spectrometry predicts severity of patients with glutaric aciduria type 2.
    Journal of chromatography. B, Analytical technologies in the biomedical and life sciences, 2010, Jun-15, Volume: 878, Issue:20

    Topics: Carnitine; Case-Control Studies; Cells, Cultured; Fibroblasts; Humans; Molecular Structure; Multiple Acyl Coenzyme A Dehydrogenase Deficiency; Spectrometry, Mass, Electrospray Ionization

2010
Altered brain phospholipid and acylcarnitine profiles in propionic acid infused rodents: further development of a potential model of autism spectrum disorders.
    Journal of neurochemistry, 2010, Volume: 113, Issue:2

    Topics: Animals; Behavior, Animal; Brain; Carnitine; Child; Child Development Disorders, Pervasive; Chromatography, Thin Layer; Disease Models, Animal; Gas Chromatography-Mass Spectrometry; Humans; Injections, Intraventricular; Male; Motor Activity; Phospholipids; Propionates; Rats; Rats, Long-Evans; Spectrometry, Mass, Electrospray Ionization; Stereotyped Behavior

2010
Useful second-tier tests in expanded newborn screening of isovaleric acidemia and methylmalonic aciduria.
    Journal of inherited metabolic disease, 2010, Volume: 33, Issue:Suppl 2

    Topics: Amino Acid Metabolism, Inborn Errors; Biomarkers; Blood Specimen Collection; Carnitine; Case-Control Studies; Chromatography, Liquid; False Positive Reactions; Gas Chromatography-Mass Spectrometry; Glycine; Humans; Infant, Newborn; Isovaleryl-CoA Dehydrogenase; Japan; Lactic Acid; Metabolism, Inborn Errors; Methylmalonic Acid; Neonatal Screening; Pilot Projects; Predictive Value of Tests; Retrospective Studies; Severity of Illness Index; Tandem Mass Spectrometry

2010
Pre-exercise medium-chain triglyceride application prevents acylcarnitine accumulation in skeletal muscle from very-long-chain acyl-CoA-dehydrogenase-deficient mice.
    Journal of inherited metabolic disease, 2010, Volume: 33, Issue:3

    Topics: Acyl Coenzyme A; Acyl-CoA Dehydrogenase, Long-Chain; Animal Feed; Animals; Carnitine; Lipids; Mice; Mice, Knockout; Muscle, Skeletal; Oxazines; Oxygen; Physical Conditioning, Animal; Triglycerides

2010
Atypical antipsychotics rapidly and inappropriately switch peripheral fuel utilization to lipids, impairing metabolic flexibility in rodents.
    Schizophrenia bulletin, 2012, Volume: 38, Issue:1

    Topics: Animals; Antipsychotic Agents; Benzodiazepines; Carnitine; Clozapine; Energy Metabolism; Fatty Acids, Nonesterified; Female; Haloperidol; Insulin Resistance; Lipolysis; Male; Malonyl Coenzyme A; Mice; Olanzapine; Piperazines; Rats; Rats, Sprague-Dawley; Risperidone; Thiazoles; Vitamin B Complex

2012
Metabolic profiling of muscle contraction in lean compared with obese rodents.
    American journal of physiology. Regulatory, integrative and comparative physiology, 2010, Volume: 299, Issue:3

    Topics: Acetyl-CoA Carboxylase; Animals; Biological Transport; Carnitine; Glucose; Glycogen; Lactic Acid; Lipids; Malonyl Coenzyme A; Muscle Contraction; Muscle, Skeletal; Obesity; Pyruvic Acid; Rats; Rats, Zucker; Sciatic Nerve; Triglycerides

2010
Carnitine palmitoyltransferase 2: New insights on the substrate specificity and implications for acylcarnitine profiling.
    Biochimica et biophysica acta, 2010, Volume: 1802, Issue:9

    Topics: Acyl Coenzyme A; Carnitine; Carnitine O-Palmitoyltransferase; Catalysis; Humans; Kinetics; Metabolome; Organisms, Genetically Modified; Osmolar Concentration; Saccharomyces cerevisiae; Substrate Specificity

2010
Carnitine palmitoyltransferase 2 deficiency: the time-course of blood and urinary acylcarnitine levels during initial L-carnitine supplementation.
    The Tohoku journal of experimental medicine, 2010, Volume: 221, Issue:3

    Topics: Acetylcarnitine; Amino Acid Metabolism, Inborn Errors; Amino Acids; Blood Chemical Analysis; Carnitine; Carnitine O-Palmitoyltransferase; Child, Preschool; Female; Follow-Up Studies; Humans; Infant; Lipid Metabolism, Inborn Errors; Reye Syndrome; Time Factors; Treatment Outcome; Vitamin B Complex

2010
Tandem mass spectrometry screening for very long-chain acyl-CoA dehydrogenase deficiency: the value of second-tier enzyme testing.
    The Journal of pediatrics, 2010, Volume: 157, Issue:4

    Topics: Acyl-CoA Dehydrogenase, Long-Chain; Carnitine; Clinical Enzyme Tests; Humans; Infant, Newborn; Metabolism, Inborn Errors; Neonatal Screening; Tandem Mass Spectrometry

2010
Rapid determination of C4-acylcarnitine and C5-acylcarnitine isomers in plasma and dried blood spots by UPLC-MS/MS as a second tier test following flow-injection MS/MS acylcarnitine profile analysis.
    Molecular genetics and metabolism, 2010, Volume: 101, Issue:1

    Topics: Carnitine; Chromatography, Liquid; Humans; Infant, Newborn; Isomerism; Neonatal Screening; Tandem Mass Spectrometry

2010
The effects of long- or medium-chain fat diets on glucose tolerance and myocellular content of lipid intermediates in rats.
    Obesity (Silver Spring, Md.), 2011, Volume: 19, Issue:4

    Topics: Analysis of Variance; Animals; Blood Glucose; Blotting, Western; Body Weight; Carnitine; Dietary Fats; Diglycerides; Energy Metabolism; Fatty Acids; Glucose Intolerance; Glucose Tolerance Test; Insulin; Insulin Resistance; Lipid Peroxidation; Male; Muscle, Skeletal; Rats; Rats, Wistar; Triglycerides

2011
Postprandial changes of amino acid and acylcarnitine concentrations in dried blood samples.
    Journal of inherited metabolic disease, 2010, Volume: 33, Issue:Suppl 2

    Topics: Adult; Amino Acids; Biomarkers; Blood Specimen Collection; Carnitine; False Negative Reactions; False Positive Reactions; Humans; Infant, Newborn; Metabolism, Inborn Errors; Middle Aged; Neonatal Screening; Postprandial Period; Predictive Value of Tests; Up-Regulation; Young Adult

2010
Post-mortem MRI reveals CPT2 deficiency after sudden infant death.
    European journal of pediatrics, 2010, Volume: 169, Issue:12

    Topics: Carnitine; Carnitine O-Palmitoyltransferase; Cause of Death; Diagnosis; Fatty Liver; Hepatomegaly; Humans; Infant; Magnetic Resonance Imaging; Male; Metabolism, Inborn Errors; Postmortem Changes; Sudden Infant Death

2010
Clinical and biochemical improvement of very long-chain acyl-CoA dehydrogenase deficiency in pregnancy.
    Journal of perinatology : official journal of the California Perinatal Association, 2010, Volume: 30, Issue:8

    Topics: Acyl-CoA Dehydrogenase, Long-Chain; Carnitine; Female; Humans; Infant, Newborn; Lipid Metabolism, Inborn Errors; Male; Pregnancy; Pregnancy Complications; Prenatal Care; Rhabdomyolysis; Term Birth; Young Adult

2010
Global analysis of circulating metabolites in hibernating ground squirrels.
    Comparative biochemistry and physiology. Part D, Genomics & proteomics, 2010, Volume: 5, Issue:4

    Topics: Animals; Carnitine; Cholesterol Esters; Chromatography, Liquid; Female; Hibernation; Lysophospholipids; Male; Mass Spectrometry; Metabolomics; Sciuridae; Seasons; Sphingosine

2010
Genotype-phenotype correlations in a large series of patients with muscle type CPT II deficiency.
    Neurological research, 2011, Volume: 33, Issue:1

    Topics: Adolescent; Adult; Carnitine; Carnitine O-Palmitoyltransferase; Child; Child, Preschool; Female; Genetic Association Studies; Humans; Male; Metabolism, Inborn Errors; Middle Aged; Muscle, Skeletal; Mutation

2011
Urgent metabolic service improves survival in long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency detected by symptomatic identification and pilot newborn screening.
    Journal of inherited metabolic disease, 2011, Volume: 34, Issue:1

    Topics: 3-Hydroxyacyl CoA Dehydrogenases; Adolescent; Carnitine; Child; Child, Preschool; Cohort Studies; Desiccation; DNA Mutational Analysis; Emergency Service, Hospital; Female; Gas Chromatography-Mass Spectrometry; Humans; Infant; Infant, Newborn; Intensive Care Units, Neonatal; Long-Chain-3-Hydroxyacyl-CoA Dehydrogenase; Male; Metabolism, Inborn Errors; Neonatal Screening; Physical Examination; Pilot Projects; Polymorphism, Restriction Fragment Length; Survival; Time Factors; Urinalysis

2011
Role of FOXA and Sp1 in mitochondrial acylcarnitine carrier gene expression in different cell lines.
    Biochemical and biophysical research communications, 2011, Jan-07, Volume: 404, Issue:1

    Topics: Binding Sites; Carnitine; Gene Expression Regulation, Enzymologic; HEK293 Cells; Hepatocyte Nuclear Factor 3-alpha; Humans; Membrane Transport Proteins; Mitochondria; Mutation; Promoter Regions, Genetic; Sp1 Transcription Factor; Transcription, Genetic

2011
A high-fat diet elicits differential responses in genes coordinating oxidative metabolism in skeletal muscle of lean and obese individuals.
    The Journal of clinical endocrinology and metabolism, 2011, Volume: 96, Issue:3

    Topics: Adolescent; Adult; Blood Glucose; Body Mass Index; Carnitine; Diet; Dietary Fats; Fatty Acids, Nonesterified; Female; Gene Expression Regulation; Humans; Insulin; Ion Channels; Lipid Metabolism; Male; Mitochondrial Proteins; Muscle, Skeletal; Obesity; Oxidation-Reduction; PPAR alpha; Pyruvate Dehydrogenase (Lipoamide); RNA, Messenger; Spectrometry, Mass, Electrospray Ionization; Uncoupling Protein 3; Young Adult

2011
Evaluation of valproate effects on acylcarnitine in epileptic children by LC-MS/MS.
    Brain & development, 2011, Volume: 33, Issue:10

    Topics: Adolescent; Anticonvulsants; Carnitine; Child; Child, Preschool; Chromatography, Liquid; Epilepsy; Female; Humans; Infant; Linear Models; Longitudinal Studies; Male; Statistics, Nonparametric; Tandem Mass Spectrometry; Valproic Acid

2011
Long-chain acylcarnitine deficiency in patients with chronic fatigue syndrome. Potential involvement of altered carnitine palmitoyltransferase-I activity.
    Journal of internal medicine, 2011, Volume: 270, Issue:1

    Topics: Adult; Aged; Carnitine; Carnitine O-Palmitoyltransferase; Epidemiologic Methods; Fatigue Syndrome, Chronic; Female; Humans; Male; Middle Aged

2011
Acylcarnitines: analysis in plasma and whole blood using tandem mass spectrometry.
    Methods in molecular biology (Clifton, N.J.), 2011, Volume: 708

    Topics: Analytic Sample Preparation Methods; Blood Chemical Analysis; Blood Specimen Collection; Carnitine; Esters; Filtration; Humans; Paper; Plasma; Reference Standards; Reproducibility of Results; Tandem Mass Spectrometry; Time Factors

2011
[Blood spot carnitine and acylcarnitine in newborn to adolescence: measured by tandem mass spectrometry].
    Zhonghua er ke za zhi = Chinese journal of pediatrics, 2010, Volume: 48, Issue:12

    Topics: Adolescent; Carnitine; Child; Child, Preschool; Female; Humans; Infant; Infant, Newborn; Male; Tandem Mass Spectrometry

2010
Improved tandem mass spectrometry (MS/MS) derivatized method for the detection of tyrosinemia type I, amino acids and acylcarnitine disorders using a single extraction process.
    Clinica chimica acta; international journal of clinical chemistry, 2011, May-12, Volume: 412, Issue:11-12

    Topics: Amino Acids; Carnitine; Chemical Fractionation; Cost-Benefit Analysis; Heptanoates; Humans; Hydrazones; Infant, Newborn; Tandem Mass Spectrometry; Time Factors; Tyrosinemias

2011
Retrospective review of Japanese sudden unexpected death in infancy: the importance of metabolic autopsy and expanded newborn screening.
    Molecular genetics and metabolism, 2011, Volume: 102, Issue:4

    Topics: Acyl-CoA Dehydrogenase, Long-Chain; Carnitine; Carnitine Acyltransferases; Carnitine O-Palmitoyltransferase; Case-Control Studies; Female; Haplotypes; Humans; Infant; Infant, Newborn; Japan; Liver; Male; Mitochondrial Trifunctional Protein; Multienzyme Complexes; Mutation, Missense; Neonatal Screening; Retrospective Studies; Sudden Infant Death

2011
Clinical, biochemical and genetic analyses in two Korean patients with medium-chain acyl-CoA dehydrogenase deficiency.
    The Korean journal of laboratory medicine, 2011, Volume: 31, Issue:1

    Topics: Acyl-CoA Dehydrogenase; Asian People; Base Sequence; Biomarkers; Carnitine; DNA Mutational Analysis; Exons; Female; Gene Deletion; Heterozygote; Humans; Infant, Newborn; Lipid Metabolism, Inborn Errors; Male; Mutation; Neonatal Screening; Republic of Korea; Tandem Mass Spectrometry

2011
Bioactives from bitter melon enhance insulin signaling and modulate acyl carnitine content in skeletal muscle in high-fat diet-fed mice.
    The Journal of nutritional biochemistry, 2011, Volume: 22, Issue:11

    Topics: Animals; Carnitine; Cells, Cultured; Diet, High-Fat; Glucose; Insulin; Male; Mice; Momordica charantia; Muscle Fibers, Skeletal; Muscle, Skeletal; Plant Extracts; Signal Transduction

2011
Mutations of ACADS gene associated with short-chain acyl-coenzyme A dehydrogenase deficiency.
    Annals of clinical and laboratory science, 2011,Fall, Volume: 41, Issue:1

    Topics: Acyl-CoA Dehydrogenase; Base Sequence; Carnitine; DNA Mutational Analysis; Female; Genetic Predisposition to Disease; Homozygote; Humans; Infant, Newborn; Lipid Metabolism, Inborn Errors; Molecular Sequence Data; Mutation

2011
Clinical validation of cutoff target ranges in newborn screening of metabolic disorders by tandem mass spectrometry: a worldwide collaborative project.
    Genetics in medicine : official journal of the American College of Medical Genetics, 2011, Volume: 13, Issue:3

    Topics: Amino Acids; Carnitine; Humans; Infant, Newborn; International Cooperation; Metabolic Diseases; Neonatal Screening; Reference Values; Sensitivity and Specificity; Software; Tandem Mass Spectrometry

2011
Carnitine and coenzyme Q10 levels in individuals with Prader-Willi syndrome.
    American journal of medical genetics. Part A, 2011, Volume: 155A, Issue:3

    Topics: Carnitine; Case-Control Studies; Child; Female; Humans; Male; Prader-Willi Syndrome; Siblings; Ubiquinone

2011
Ultra-high performance liquid chromatography tandem mass spectrometry for comprehensive analysis of urinary acylcarnitines.
    Analytica chimica acta, 2011, Mar-09, Volume: 689, Issue:1

    Topics: Adult; Carnitine; Chromatography, High Pressure Liquid; Humans; Lipid Metabolism; Metabolomics; Microsomes, Liver; Solid Phase Extraction; Tandem Mass Spectrometry; Young Adult

2011
Abnormally low serum acylcarnitine levels in narcolepsy patients.
    Sleep, 2011, Mar-01, Volume: 34, Issue:3

    Topics: Adult; Carnitine; Carnitine O-Palmitoyltransferase; Case-Control Studies; Female; Gene Expression; Genotype; Humans; Male; Middle Aged; Narcolepsy; Polymorphism, Single Nucleotide; Reverse Transcriptase Polymerase Chain Reaction

2011
Plasma acylcarnitines are associated with physical performance in elderly men.
    The journals of gerontology. Series A, Biological sciences and medical sciences, 2011, Volume: 66, Issue:5

    Topics: Aged; Carnitine; Gait; Hand Strength; Humans; Male; Mass Spectrometry; Motor Activity; Overweight

2011
Positive newborn screen in a normal infant of a mother with asymptomatic very long-chain Acyl-CoA dehydrogenase deficiency.
    The Journal of pediatrics, 2011, Volume: 158, Issue:6

    Topics: Acyl-CoA Dehydrogenase, Long-Chain; Carnitine; Congenital Bone Marrow Failure Syndromes; Female; Genotype; Humans; Infant, Newborn; Lipid Metabolism, Inborn Errors; Male; Metabolism, Inborn Errors; Mitochondrial Diseases; Mothers; Muscular Diseases; Neonatal Screening; Phenotype

2011
Capillary electrophoresis with contactless conductivity detection for the determination of carnitine and acylcarnitines in clinical samples.
    Journal of chromatography. B, Analytical technologies in the biomedical and life sciences, 2011, Apr-15, Volume: 879, Issue:13-14

    Topics: Carnitine; Electric Conductivity; Electrophoresis, Capillary; Humans; Hydrogen-Ion Concentration; Linear Models; Reproducibility of Results; Sensitivity and Specificity; Solid Phase Extraction

2011
Postnatal variations in blood free and acylcarnitines.
    Journal of clinical laboratory analysis, 2011, Volume: 25, Issue:2

    Topics: Carnitine; Female; Genetic Variation; Gestational Age; Humans; Infant, Newborn; Male; Metabolism, Inborn Errors; Neonatal Screening; Reference Values; Spectrometry, Mass, Electrospray Ionization; Tandem Mass Spectrometry

2011
Analysis of inborn errors of metabolism: disease spectrum for expanded newborn screening in Hong Kong.
    Chinese medical journal, 2011, Volume: 124, Issue:7

    Topics: Acids; Amino Acids; Carnitine; Hong Kong; Humans; Infant, Newborn; Metabolism, Inborn Errors; Neonatal Screening; Tandem Mass Spectrometry

2011
Metabolic remodeling of human skeletal myocytes by cocultured adipocytes depends on the lipolytic state of the system.
    Diabetes, 2011, Volume: 60, Issue:7

    Topics: Adipocytes; Carnitine; Coculture Techniques; Fatty Acids, Nonesterified; Female; Gene Expression; Glucose; Humans; Insulin; Insulin Resistance; Lipolysis; Muscle Fibers, Skeletal; Obesity; Thinness; Triglycerides

2011
Expanded molecular features of carnitine acyl-carnitine translocase (CACT) deficiency by comprehensive molecular analysis.
    Molecular genetics and metabolism, 2011, Volume: 103, Issue:4

    Topics: Adult; Base Sequence; Carnitine; Carnitine Acyltransferases; Child, Preschool; Comparative Genomic Hybridization; Exons; Female; Humans; Infant; Male; Membrane Transport Proteins; Molecular Sequence Data; Mutation

2011
Fatal viral infection-associated encephalopathy in two Chinese boys: a genetically determined risk factor of thermolabile carnitine palmitoyltransferase II variants.
    Journal of human genetics, 2011, Volume: 56, Issue:8

    Topics: Amino Acid Substitution; Base Sequence; Carnitine; Carnitine O-Palmitoyltransferase; Child, Preschool; DNA Mutational Analysis; Encephalitis, Viral; Enzyme Stability; Family Health; Fatal Outcome; Female; Genetic Predisposition to Disease; Genotype; Humans; Influenza, Human; Male; Molecular Sequence Data; Mutation; Pedigree; Risk Factors; Temperature

2011
Urinary acylcarnitines are altered in human kidney cancer.
    International journal of cancer, 2012, Jun-15, Volume: 130, Issue:12

    Topics: Animals; Biomarkers, Tumor; Carnitine; Cell Line, Tumor; Humans; Kidney; Kidney Neoplasms; Metabolomics; Mice; Neoplasm Grading; Neoplasm Transplantation; NF-kappa B; Transplantation, Heterologous

2012
Altered carnitine metabolism in dialysis patients with reduced physical function may be due to dysfunctional fatty acid oxidation.
    Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association, 2012, Volume: 27, Issue:1

    Topics: Activities of Daily Living; Aged; Anemia; Carnitine; Cross-Sectional Studies; Erythrocytes; Erythropoietin; Fatty Acids; Female; Follow-Up Studies; Humans; Lipid Metabolism; Male; Oxidation-Reduction; Prognosis; Quality of Life; Renal Dialysis

2012
Rare magnetic resonance imaging findings in medium-chain acyl-coenzyme A dehydrogenase deficiency.
    Pediatric neurology, 2011, Volume: 45, Issue:3

    Topics: Acyl-CoA Dehydrogenase; Anticonvulsants; Basal Ganglia; Brain; Brain Ischemia; Carnitine; Cerebral Infarction; Child, Preschool; Glasgow Coma Scale; Humans; Lipid Metabolism, Inborn Errors; Liver Function Tests; Magnetic Resonance Imaging; Male; Respiration, Artificial; Seizures; Valproic Acid

2011
Discovery of sexual dimorphisms in metabolic and genetic biomarkers.
    PLoS genetics, 2011, Volume: 7, Issue:8

    Topics: Adult; Aged; Aged, 80 and over; Amino Acids; Carnitine; Female; Genetic Markers; Genome-Wide Association Study; Glycine; Humans; Lipids; Male; Metabolome; Middle Aged; Polymorphism, Single Nucleotide; Sex Characteristics

2011
Tandem mass spectrometry findings at autopsy for detection of metabolic disease in infant deaths: postmortem changes and confounding factors.
    Journal of clinical pathology, 2011, Volume: 64, Issue:11

    Topics: Autopsy; Bile; Carnitine; Humans; Infant; Infant, Newborn; Liver; Metabolic Diseases; Organ Size; Postmortem Changes; Sudden Infant Death; Tandem Mass Spectrometry

2011
Cardiac involvement in inflammatory bowel disease: role of acylcarnitine esters.
    La Clinica terapeutica, 2011, Volume: 162, Issue:4

    Topics: Adult; Cardiomyopathy, Dilated; Carnitine; Case-Control Studies; Female; Heart Ventricles; Humans; Inflammatory Bowel Diseases; Malabsorption Syndromes; Male; Middle Aged; Myocardium; Ultrasonography; Young Adult

2011
Severe dysfunction of respiratory chain and cholesterol metabolism in Atp7b(-/-) mice as a model for Wilson disease.
    Biochimica et biophysica acta, 2011, Volume: 1812, Issue:12

    Topics: Aconitate Hydratase; Adenosine Triphosphatases; Alanine; Animals; Brain; Carnitine; Cation Transport Proteins; Cholesterol; Copper; Copper-Transporting ATPases; Disease Models, Animal; Electron Transport; Electron Transport Chain Complex Proteins; Gene Knockout Techniques; Glutathione; Hepatolenticular Degeneration; Humans; Liver; Mice; Mice, 129 Strain; Mice, Knockout; Mitochondria; Oxidative Stress; Superoxide Dismutase

2011
Hepatic and muscular effects of different dietary fat content in VLCAD deficient mice.
    Molecular genetics and metabolism, 2011, Volume: 104, Issue:4

    Topics: Acyl-CoA Dehydrogenase, Long-Chain; Animals; Carnitine; Congenital Bone Marrow Failure Syndromes; Diet; Dietary Fats; Energy Metabolism; Glycogen; Lipid Metabolism; Lipid Metabolism, Inborn Errors; Lipids; Lipogenesis; Liver; Mice; Mice, 129 Strain; Mice, Inbred C57BL; Mice, Knockout; Mitochondrial Diseases; Muscle, Skeletal; Muscular Diseases; Physical Exertion; Up-Regulation

2011
An improved LC-MS/MS method for the detection of classic and low excretor glutaric acidemia type 1.
    Journal of inherited metabolic disease, 2012, Volume: 35, Issue:3

    Topics: Amino Acid Metabolism, Inborn Errors; Brain Diseases, Metabolic; Carnitine; Case-Control Studies; Chromatography, Liquid; Follow-Up Studies; Glutaryl-CoA Dehydrogenase; Humans; Ions; Predictive Value of Tests; Reproducibility of Results; Tandem Mass Spectrometry

2012
Analysis of plasma ghrelin in patients with medium-chain acyl-CoA dehydrogenase deficiency and glutaric aciduria type II.
    European journal of endocrinology, 2012, Volume: 166, Issue:2

    Topics: Acyl-CoA Dehydrogenase; Adult; Blood Chemical Analysis; Carnitine; Case-Control Studies; Child; Child, Preschool; Chromatography, High Pressure Liquid; Female; Ghrelin; Humans; Lipid Metabolism, Inborn Errors; Multiple Acyl Coenzyme A Dehydrogenase Deficiency; Protein Processing, Post-Translational; Young Adult

2012
Bile acid sequestration reduces plasma glucose levels in db/db mice by increasing its metabolic clearance rate.
    PloS one, 2011, Volume: 6, Issue:11

    Topics: Animals; Bile Acids and Salts; Blood Glucose; Carbon Isotopes; Carnitine; Diabetes Mellitus, Experimental; Glucose; Liver; Metabolic Clearance Rate; Mice; Mice, Knockout; Muscle, Skeletal

2011
Combined extraction of acyl carnitines and 26:0 lysophosphatidylcholine from dried blood spots: prospective newborn screening for X-linked adrenoleukodystrophy.
    Molecular genetics and metabolism, 2012, Volume: 105, Issue:3

    Topics: Adrenoleukodystrophy; Carnitine; Chromatography, Liquid; Dried Blood Spot Testing; Humans; Infant, Newborn; Lysophosphatidylcholines; Mass Spectrometry; Molecular Diagnostic Techniques; Neonatal Screening; Peroxisomal Disorders; Peroxisomes

2012
Brown adipose tissue mitochondria oxidizing fatty acids generate high levels of reactive oxygen species irrespective of the uncoupling protein-1 activity state.
    Biochimica et biophysica acta, 2012, Volume: 1817, Issue:3

    Topics: Adipose Tissue, Brown; Animals; Carnitine; Fatty Acids; Hydrogen Peroxide; Ion Channels; Membrane Potential, Mitochondrial; Mitochondria; Mitochondrial Proteins; Nigericin; Onium Compounds; Oxidation-Reduction; Rats; Reactive Oxygen Species; Rotenone; Uncoupling Protein 1

2012
Deletion of CaMKK2 from the liver lowers blood glucose and improves whole-body glucose tolerance in the mouse.
    Molecular endocrinology (Baltimore, Md.), 2012, Volume: 26, Issue:2

    Topics: Adenylate Kinase; Animals; Antigens, Neoplasm; Blood Glucose; Calcium-Calmodulin-Dependent Protein Kinase Kinase; Carnitine; Catecholamines; Cells, Cultured; Diet, High-Fat; Eating; Fatty Liver; Gene Knockout Techniques; Gluconeogenesis; Glucose; Glucose Intolerance; Hepatocytes; Homeostasis; Intra-Abdominal Fat; Lipid Metabolism; Liver; Mice; Mice, Knockout; Phospholipases A1; Primary Cell Culture; Signal Transduction

2012
Postmortem tandem mass spectrometry profiling for detection of infection in unexpected infant death.
    Forensic science, medicine, and pathology, 2012, Volume: 8, Issue:3

    Topics: Autopsy; Bile; Biomarkers; Carnitine; Cause of Death; Communicable Diseases; Forensic Toxicology; Humans; Infant; Infant Mortality; Infant, Newborn; Linear Models; London; Postmortem Changes; Predictive Value of Tests; Reference Standards; Retrospective Studies; Risk Factors; Sudden Infant Death; Tandem Mass Spectrometry

2012
Metabolic decompensation in methylmalonic aciduria: which biochemical parameters are discriminative?
    Journal of inherited metabolic disease, 2012, Volume: 35, Issue:5

    Topics: Acid-Base Equilibrium; Acidosis; Adolescent; Adult; Amino Acid Metabolism, Inborn Errors; Ammonia; Bicarbonates; Carnitine; Child; Child, Preschool; Female; Follow-Up Studies; Humans; Infant; Infant, Newborn; Male; Young Adult

2012
Analysis of acylcarnitine levels by tandem mass spectrometry in epileptic children receiving valproate and oxcarbazepine.
    Epileptic disorders : international epilepsy journal with videotape, 2011, Volume: 13, Issue:4

    Topics: Adolescent; Anticonvulsants; Carbamazepine; Carnitine; Child; Child, Preschool; Chromatography, High Pressure Liquid; Epilepsy; Female; Humans; Male; Oxcarbazepine; Prospective Studies; Seizures; Tandem Mass Spectrometry; Valproic Acid

2011
Quantification of acylglycines in human urine by HPLC electrospray ionization-tandem mass spectrometry and the establishment of pediatric reference interval in local Chinese.
    Talanta, 2012, Jan-15, Volume: 88

    Topics: 1-Butanol; Acylation; Adolescent; Asian People; Calibration; Carnitine; Child; Child, Preschool; Chromatography, High Pressure Liquid; Deuterium; Female; Glycine; Humans; Infant; Infant, Newborn; Male; Metabolism, Inborn Errors; Reference Values; Sensitivity and Specificity; Solid Phase Extraction; Spectrometry, Mass, Electrospray Ionization; Tandem Mass Spectrometry

2012
Metabolomic profiling of fatty acid and amino acid metabolism in youth with obesity and type 2 diabetes: evidence for enhanced mitochondrial oxidation.
    Diabetes care, 2012, Volume: 35, Issue:3

    Topics: Absorptiometry, Photon; Adolescent; Amino Acids; Carnitine; Diabetes Mellitus, Type 2; Fasting; Fatty Acids; Female; Humans; Male; Obesity

2012
Metabolic profiles predict adverse events after coronary artery bypass grafting.
    The Journal of thoracic and cardiovascular surgery, 2012, Volume: 143, Issue:4

    Topics: Aged; Biomarkers; Carnitine; Coronary Artery Bypass; Coronary Artery Disease; Energy Metabolism; Female; Humans; Kaplan-Meier Estimate; Ketones; Male; Mass Spectrometry; Metabolomics; Middle Aged; Multivariate Analysis; Myocardial Infarction; North Carolina; Odds Ratio; Principal Component Analysis; Proportional Hazards Models; Reoperation; Risk Assessment; Risk Factors; Time Factors; Treatment Outcome

2012
Serum metabolomics reveals the deregulation of fatty acids metabolism in hepatocellular carcinoma and chronic liver diseases.
    Analytical and bioanalytical chemistry, 2012, Volume: 403, Issue:1

    Topics: Carcinoma, Hepatocellular; Carnitine; Chromatography, Liquid; Chronic Disease; Fatty Acids; Humans; Liver Diseases; Liver Neoplasms; Mass Spectrometry; Metabolomics

2012
Identification by site-directed mutagenesis of a hydrophobic binding site of the mitochondrial carnitine/acylcarnitine carrier involved in the interaction with acyl groups.
    Biochimica et biophysica acta, 2012, Volume: 1817, Issue:5

    Topics: Acylation; Animals; Binding Sites; Carnitine; Carnitine Acyltransferases; Computational Biology; Enzyme Inhibitors; Hydrophobic and Hydrophilic Interactions; Kinetics; Mitochondria; Models, Molecular; Mutagenesis, Site-Directed; Mutant Proteins; Palmitoylcarnitine; Protein Binding; Rats; Time Factors

2012
Long-term correction of very long-chain acyl-coA dehydrogenase deficiency in mice using AAV9 gene therapy.
    Molecular therapy : the journal of the American Society of Gene Therapy, 2012, Volume: 20, Issue:6

    Topics: Acyl-CoA Dehydrogenase, Long-Chain; Animals; Carnitine; Congenital Bone Marrow Failure Syndromes; Dependovirus; Gene Expression; Genetic Therapy; Genetic Vectors; Lipid Metabolism; Lipid Metabolism, Inborn Errors; Liver; Mice; Mice, Knockout; Mitochondrial Diseases; Muscle, Skeletal; Muscular Diseases; Phenotype; Tissue Distribution; Transduction, Genetic

2012
Intrahepatic diacylglycerol content is associated with hepatic insulin resistance in obese subjects.
    Gastroenterology, 2012, Volume: 142, Issue:7

    Topics: Adult; Carnitine; Ceramides; Diglycerides; Female; Glucose; Humans; Insulin Resistance; Liver; Male; Obesity, Morbid

2012
Serum metabolomic profiles from patients with acute kidney injury: a pilot study.
    Journal of chromatography. B, Analytical technologies in the biomedical and life sciences, 2012, Apr-15, Volume: 893-894

    Topics: Acute Kidney Injury; Adult; Amino Acids; Biomarkers; Carnitine; Case-Control Studies; Chromatography, High Pressure Liquid; Discriminant Analysis; Female; Humans; Male; Mass Spectrometry; Metabolic Networks and Pathways; Metabolome; Metabolomics; Pilot Projects

2012
Serum metabolite concentrations and decreased GFR in the general population.
    American journal of kidney diseases : the official journal of the National Kidney Foundation, 2012, Volume: 60, Issue:2

    Topics: Carnitine; Creatinine; Cross-Sectional Studies; Cystatin C; Female; Glomerular Filtration Rate; Humans; Kidney Function Tests; Male; Metabolomics; Middle Aged; Reference Values; Renal Insufficiency, Chronic

2012
Tissue-specific splicing of an Ndufs6 gene-trap insertion generates a mitochondrial complex I deficiency-specific cardiomyopathy.
    Proceedings of the National Academy of Sciences of the United States of America, 2012, Apr-17, Volume: 109, Issue:16

    Topics: Adenosine Triphosphate; Animals; Animals, Newborn; Blotting, Western; Cardiomyopathies; Carnitine; Cell Line; Electron Transport Complex I; Female; Gene Expression Profiling; Heart; Humans; In Vitro Techniques; Kaplan-Meier Estimate; Male; Mice; Mice, 129 Strain; Mice, Inbred C57BL; Mice, Knockout; Microscopy, Electron; Mitochondria; Mitochondrial Diseases; Mutagenesis, Insertional; Myocardium; NADH Dehydrogenase; Reverse Transcriptase Polymerase Chain Reaction; RNA Splicing

2012
Analysis of acylcarnitine profiles in umbilical cord blood and during the early neonatal period by electrospray ionization tandem mass spectrometry.
    Brazilian journal of medical and biological research = Revista brasileira de pesquisas medicas e biologicas, 2012, Volume: 45, Issue:6

    Topics: Brazil; Breast Feeding; Carnitine; Dried Blood Spot Testing; Fatty Acids; Female; Fetal Blood; Humans; Infant, Newborn; Male; Neonatal Screening; Spectrometry, Mass, Electrospray Ionization; Statistics, Nonparametric; Tandem Mass Spectrometry

2012
Changing metabolic signatures of amino acids and lipids during the prediabetic period in a pig model with impaired incretin function and reduced β-cell mass.
    Diabetes, 2012, Volume: 61, Issue:8

    Topics: Aging; Amino Acids; Animals; Animals, Genetically Modified; Biomarkers; Carnitine; Diglycerides; Disease Progression; Glucose Tolerance Test; Incretins; Insulin; Insulin Secretion; Insulin-Secreting Cells; Lipids; Lysophosphatidylcholines; Phospholipid Ethers; Prediabetic State; Receptors, Gastrointestinal Hormone; Sphingomyelins; Swine; Transcriptome

2012
MCAD deficiency in Denmark.
    Molecular genetics and metabolism, 2012, Volume: 106, Issue:2

    Topics: Acyl-CoA Dehydrogenase; Alleles; Base Sequence; Carnitine; Denmark; Family; Female; Genetic Association Studies; Genotype; Humans; Incidence; Infant; Infant, Newborn; Lipid Metabolism, Inborn Errors; Male; Mutation; Neonatal Screening; Phenotype; Tandem Mass Spectrometry

2012
Muscle-specific deletion of carnitine acetyltransferase compromises glucose tolerance and metabolic flexibility.
    Cell metabolism, 2012, May-02, Volume: 15, Issue:5

    Topics: Acetyl Coenzyme A; Acetylcarnitine; Animals; Carbon; Carnitine; Carnitine O-Acetyltransferase; Cells, Cultured; Energy Metabolism; Fatty Acids; Glucose; Glucose Tolerance Test; Humans; Insulin; Insulin Resistance; Mice; Mice, Knockout; Mitochondria; Muscle Fibers, Skeletal

2012
Diagnosis of a patient with a kinetic variant of medium and short-chain 3-hydroxyacyl-CoA dehydrogenase deficiency by newborn screening.
    Molecular genetics and metabolism, 2012, Volume: 106, Issue:3

    Topics: 3-Hydroxyacyl CoA Dehydrogenases; Carnitine; Child, Preschool; Genetic Variation; Humans; Infant, Newborn; Metabolism, Inborn Errors; Molecular Sequence Data; Mutation; Neonatal Screening

2012
Mutations at the flavin binding site of ETF:QO yield a MADD-like severe phenotype in Drosophila.
    Biochimica et biophysica acta, 2012, Volume: 1822, Issue:8

    Topics: Alleles; Amino Acid Sequence; Animals; Binding Sites; Carnitine; Drosophila; Electron-Transferring Flavoproteins; Flavin-Adenine Dinucleotide; Flavins; Genotype; Models, Molecular; Molecular Sequence Data; Multiple Acyl Coenzyme A Dehydrogenase Deficiency; Mutation; Phenotype

2012
Muscle or liver-specific Sirt3 deficiency induces hyperacetylation of mitochondrial proteins without affecting global metabolic homeostasis.
    Scientific reports, 2012, Volume: 2

    Topics: Acetylation; Adenosine Triphosphate; Animals; Blotting, Western; Carnitine; Energy Metabolism; Female; Gene Expression; Glutathione; Homeostasis; Liver; Male; Mice; Mice, Inbred C57BL; Mice, Knockout; Mitochondria; Mitochondrial Proteins; Muscle, Skeletal; Oxidation-Reduction; Reactive Oxygen Species; Reverse Transcriptase Polymerase Chain Reaction; Sirtuin 3; Superoxide Dismutase

2012
Metabolic autopsy with postmortem cultured fibroblasts in sudden unexpected death in infancy: diagnosis of mitochondrial respiratory chain disorders.
    Molecular genetics and metabolism, 2012, Volume: 106, Issue:4

    Topics: Autopsy; Carnitine; Cells, Cultured; Electron Transport; Enzyme Assays; Female; Fibroblasts; Humans; Infant; Infant, Newborn; Liver; Male; Mitochondrial Diseases; Myocardium; Postmortem Changes; Sudden Infant Death

2012
Increases in bioavailability of poorly absorbed drug by acylcarnitine.
    Journal of pharmaceutical sciences, 2012, Volume: 101, Issue:9

    Topics: Administration, Oral; Animals; ATP Binding Cassette Transporter, Subfamily B, Member 1; Biological Availability; Carnitine; Claudin-4; Colon; Fluorescent Dyes; Injections, Intravenous; Intestinal Absorption; Isoquinolines; Jejunum; L-Lactate Dehydrogenase; Laurates; Male; Palmitoylcarnitine; Rats; Rats, Wistar

2012
[Hepatotoxicity of perfluorooctanoic acid in human hepatocytes using metabonomics].
    Se pu = Chinese journal of chromatography, 2012, Volume: 30, Issue:2

    Topics: Caprylates; Carnitine; Cell Line; Chromatography, High Pressure Liquid; Fluorocarbons; Hepatocytes; Humans; Mass Spectrometry; Metabolomics

2012
Characterization of the molecular spectrum of Medium-Chain Acyl-CoA Dehydrogenase Deficiency in a Greek newborns cohort: identification of a novel variant.
    Clinical biochemistry, 2012, Volume: 45, Issue:15

    Topics: Acyl-CoA Dehydrogenase; Alleles; Base Sequence; Carnitine; Genetic Association Studies; Greece; Humans; Infant, Newborn; Mitochondrial Diseases; Molecular Sequence Data; Neonatal Screening; Polymorphism, Restriction Fragment Length; Prevalence; Sequence Analysis, DNA

2012
Thyroid hormone stimulates hepatic lipid catabolism via activation of autophagy.
    The Journal of clinical investigation, 2012, Volume: 122, Issue:7

    Topics: Animals; Autophagy; Autophagy-Related Protein 5; Carnitine; Cell Line, Tumor; Fatty Acids; Gene Expression Profiling; Gene Knockdown Techniques; Hepatocytes; Histone Deacetylases; Humans; Ketone Bodies; Lipid Metabolism; Liver; Male; Metabolic Networks and Pathways; Metabolome; Mice; Mice, Inbred C57BL; Microtubule-Associated Proteins; Nuclear Receptor Co-Repressor 1; Oligonucleotide Array Sequence Analysis; Oxidation-Reduction; Phagosomes; Protein Binding; Receptors, Thyroid Hormone; RNA Interference; Triiodothyronine

2012
A compendium of inborn errors of metabolism mapped onto the human metabolic network.
    Molecular bioSystems, 2012, Volume: 8, Issue:10

    Topics: Acetyl Coenzyme A; Amino Acids; Brain; Carbohydrate Metabolism; Carnitine; Dried Blood Spot Testing; Fatty Acids; Genome, Human; Humans; Infant, Newborn; Lipid Metabolism; Metabolic Networks and Pathways; Metabolism, Inborn Errors; Metabolomics; Oxidation-Reduction; Tandem Mass Spectrometry

2012
Metabolomic profiling reveals mitochondrial-derived lipid biomarkers that drive obesity-associated inflammation.
    PloS one, 2012, Volume: 7, Issue:6

    Topics: Adipose Tissue; Amino Acids; Analysis of Variance; Animals; Biomarkers; Blotting, Western; Carnitine; Chromatography, Liquid; Cluster Analysis; Computational Biology; Diet; Fatty Acids; Gene Expression Regulation; Inflammation; Laurates; Macrophages; Metabolic Syndrome; Metabolomics; Mitochondria; Obesity; Principal Component Analysis; Rats; Tandem Mass Spectrometry

2012
Decreased expression of adipose CD36 and FATP1 are associated with increased plasma non-esterified fatty acids during prolonged fasting in northern elephant seal pups (Mirounga angustirostris).
    The Journal of experimental biology, 2012, Jul-15, Volume: 215, Issue:Pt 14

    Topics: Adipose Tissue; Animals; Animals, Newborn; Blotting, Western; Body Weight; Carnitine; Carnitine O-Palmitoyltransferase; CD36 Antigens; Fasting; Fatty Acid Transport Proteins; Fatty Acids, Nonesterified; Glucose; Glycerol; Intercellular Signaling Peptides and Proteins; Lipase; Lipid Metabolism; Phosphoenolpyruvate Carboxykinase (ATP); Seals, Earless; Time Factors; Triglycerides

2012
Cardiac-specific deletion of acetyl CoA carboxylase 2 prevents metabolic remodeling during pressure-overload hypertrophy.
    Circulation research, 2012, Aug-31, Volume: 111, Issue:6

    Topics: Acetyl-CoA Carboxylase; Animals; Aorta; Blotting, Western; Cardiomegaly; Carnitine; Constriction, Pathologic; Fatty Acids; Female; Fibrosis; Heart; In Vitro Techniques; Male; Malonyl Coenzyme A; Mice; Mice, 129 Strain; Mice, Inbred C57BL; Mice, Knockout; Mitochondria, Heart; Myocardium; Oxidation-Reduction; Pressure; Ventricular Remodeling

2012
Analysis of serum metabolic profiles in women with endometrial cancer and controls in a population-based case-control study.
    The Journal of clinical endocrinology and metabolism, 2012, Volume: 97, Issue:9

    Topics: Adult; Aged; Aging; Algorithms; Amino Acids; Body Mass Index; Body Weight; Carnitine; Case-Control Studies; Data Interpretation, Statistical; Endometrial Neoplasms; Fatty Acids; Female; Humans; Male; Middle Aged; Poland; Population; Quality Control

2012
Rapid and weight-independent improvement of glucose tolerance induced by a peptide designed to elicit apoptosis in adipose tissue endothelium.
    Diabetes, 2012, Volume: 61, Issue:9

    Topics: Adipose Tissue, White; Animals; Apoptosis; Blood Glucose; Carnitine; Diet, High-Fat; Energy Intake; Glucose Tolerance Test; Homeostasis; Intercellular Signaling Peptides and Proteins; Lipid Metabolism; Male; Metabolic Networks and Pathways; Mice; Mice, Inbred C57BL; Mitochondria; Peptides; Triglycerides

2012
Direct and quantitative analysis of underivatized acylcarnitines in serum and whole blood using paper spray mass spectrometry.
    Analytical and bioanalytical chemistry, 2012, Volume: 404, Issue:5

    Topics: Carnitine; Dried Blood Spot Testing; Humans; Infant, Newborn; Limit of Detection; Lipid Metabolism, Inborn Errors; Mass Spectrometry; Reproducibility of Results; Time Factors

2012
A rapid UPLC-MS/MS method for simultaneous separation of 48 acylcarnitines in dried blood spots and plasma useful as a second-tier test for expanded newborn screening.
    Analytical and bioanalytical chemistry, 2012, Volume: 404, Issue:3

    Topics: Calibration; Carnitine; Chromatography, Liquid; Dried Blood Spot Testing; Flow Injection Analysis; Humans; Infant, Newborn; Isomerism; Limit of Detection; Metabolism, Inborn Errors; Neonatal Screening; Spectrometry, Mass, Electrospray Ionization; Tandem Mass Spectrometry

2012
Functional redundancy of mitochondrial enoyl-CoA isomerases in the oxidation of unsaturated fatty acids.
    FASEB journal : official publication of the Federation of American Societies for Experimental Biology, 2012, Volume: 26, Issue:10

    Topics: Animals; Blood Glucose; Carbon-Carbon Double Bond Isomerases; Carnitine; Cells, Cultured; Chromatography, High Pressure Liquid; Dodecenoyl-CoA Isomerase; Fatty Acids, Unsaturated; Immunoblotting; Mass Spectrometry; Mice; Mice, Knockout; Mitochondria; Oxidation-Reduction; Real-Time Polymerase Chain Reaction

2012
Measurement of acylcarnitine substrate to product ratios specific to biotin-dependent carboxylases offers a combination of indicators of biotin status in humans.
    The Journal of nutrition, 2012, Volume: 142, Issue:9

    Topics: Acetyl-CoA Carboxylase; Biomarkers; Biotin; Carbon-Carbon Ligases; Carbon-Nitrogen Ligases; Carnitine; Chromatography, Liquid; Deficiency Diseases; Glutarates; Humans; Methylmalonyl-CoA Decarboxylase; Reproducibility of Results; Substrate Specificity; Tandem Mass Spectrometry

2012
Bezafibrate can be a new treatment option for mitochondrial fatty acid oxidation disorders: evaluation by in vitro probe acylcarnitine assay.
    Molecular genetics and metabolism, 2012, Volume: 107, Issue:1-2

    Topics: Bezafibrate; Carboxylic Acids; Carnitine; Cells, Cultured; Fatty Acids; Fibroblasts; Humans; Mitochondrial Diseases; Oxidation-Reduction

2012
A quantitative method for acylcarnitines and amino acids using high resolution chromatography and tandem mass spectrometry in newborn screening dried blood spot analysis.
    Journal of chromatography. B, Analytical technologies in the biomedical and life sciences, 2012, Aug-15, Volume: 903

    Topics: Amino Acids; Carnitine; Chromatography, High Pressure Liquid; Dried Blood Spot Testing; Humans; Infant, Newborn; Linear Models; Neonatal Screening; Reproducibility of Results; Sensitivity and Specificity; Tandem Mass Spectrometry

2012
Long-chain acylcarnitines regulate the hERG channel.
    PloS one, 2012, Volume: 7, Issue:7

    Topics: Carnitine; Dose-Response Relationship, Drug; Electrophysiological Phenomena; Ether-A-Go-Go Potassium Channels; Extracellular Space; HEK293 Cells; Humans; Intracellular Space

2012
Mass spectrometry images acylcarnitines, phosphatidylcholines, and sphingomyelin in MDA-MB-231 breast tumor models.
    Journal of lipid research, 2013, Volume: 54, Issue:2

    Topics: Animals; Breast Neoplasms; Carnitine; Cell Line, Tumor; Cell Transformation, Neoplastic; Humans; Mass Spectrometry; Mice; Molecular Imaging; Phosphatidylcholines; Sphingomyelins

2013
Abnormal newborn screens and acylcarnitines in HIV-exposed and ARV-exposed infants.
    The Pediatric infectious disease journal, 2013, Volume: 32, Issue:2

    Topics: Anti-Retroviral Agents; Carnitine; Case-Control Studies; HIV Infections; Humans; Infant, Newborn; Infant, Newborn, Diseases; Neonatal Screening; New York

2013
Expanded newborn screening of inborn errors of metabolism by capillary electrophoresis-electrospray ionization-mass spectrometry (CE-ESI-MS).
    Methods in molecular biology (Clifton, N.J.), 2013, Volume: 919

    Topics: Amino Acids; Carnitine; Dried Blood Spot Testing; Electrophoresis, Capillary; Humans; Infant, Newborn; Maleimides; Metabolism, Inborn Errors; Neonatal Screening; Spectrometry, Mass, Electrospray Ionization; Sulfhydryl Compounds

2013
Feasibility of electroextraction as versatile sample preconcentration for fast and sensitive analysis of urine metabolites, demonstrated on acylcarnitines.
    Electrophoresis, 2012, Volume: 33, Issue:19-20

    Topics: Carnitine; Chemical Fractionation; Chromatography, Liquid; Electrophoresis, Capillary; Equipment Design; Feasibility Studies; Humans; Limit of Detection; Linear Models; Mass Spectrometry; Metabolomics; Reproducibility of Results; Urinalysis

2012
Extraction and analysis of carnitine and acylcarnitines by electrospray ionization tandem mass spectrometry directly from dried blood and plasma spots using a novel autosampler.
    Rapid communications in mass spectrometry : RCM, 2012, Nov-15, Volume: 26, Issue:21

    Topics: Automation; Carnitine; Dried Blood Spot Testing; Humans; Spectrometry, Mass, Electrospray Ionization; Tandem Mass Spectrometry

2012
PGC-1α improves glucose homeostasis in skeletal muscle in an activity-dependent manner.
    Diabetes, 2013, Volume: 62, Issue:1

    Topics: Animals; Carnitine; Cells, Cultured; Citric Acid Cycle; Fatty Acid Synthases; Glucose; Homeostasis; Lipogenesis; Male; Mice; Muscle, Skeletal; Oxidative Phosphorylation; Peroxisome Proliferator-Activated Receptor Gamma Coactivator 1-alpha; Physical Conditioning, Animal; Sphingosine; Trans-Activators; Transcription Factors

2013
Acylcarnitine profile in thyroid disease.
    Clinical biochemistry, 2013, Volume: 46, Issue:1-2

    Topics: Adult; Aged; Carnitine; Female; Humans; Hyperthyroidism; Hypothyroidism; Male; Middle Aged; Reference Values; Thyroxine

2013
L-carnitine and long-chain acylcarnitines are positively correlated with ambulatory blood pressure in humans: the SABPA study.
    Lipids, 2013, Volume: 48, Issue:1

    Topics: Adult; Black People; Blood Pressure; Blood Pressure Monitoring, Ambulatory; Carnitine; Cross-Sectional Studies; Humans; Hypertension; Male; Middle Aged; White People

2013
Intracellular in vitro probe acylcarnitine assay for identifying deficiencies of carnitine transporter and carnitine palmitoyltransferase-1.
    Analytical and bioanalytical chemistry, 2013, Volume: 405, Issue:4

    Topics: Biological Transport; Cardiomyopathies; Carnitine; Carnitine Acyltransferases; Carnitine O-Palmitoyltransferase; Cells, Cultured; Fatty Acids; Fibroblasts; Humans; Hyperammonemia; Hypoglycemia; Lipid Metabolism, Inborn Errors; Mitochondria; Muscular Diseases; Oxidation-Reduction; Spectrometry, Mass, Electrospray Ionization; Tandem Mass Spectrometry

2013
The heritability of metabolic profiles in newborn twins.
    Heredity, 2013, Volume: 110, Issue:3

    Topics: Birth Weight; Carnitine; Female; Gestational Age; Humans; Infant, Newborn; Inheritance Patterns; Linear Models; Male; Metabolome; Neonatal Screening; Thyrotropin; Trypsinogen; Twins, Dizygotic; Twins, Monozygotic

2013
Consumption of a high β-glucan barley flour improves glucose control and fatty liver and increases muscle acylcarnitines in the Zucker diabetic fatty rat.
    European journal of nutrition, 2013, Volume: 52, Issue:7

    Topics: Adiponectin; Animals; beta-Glucans; Blood Glucose; Body Weight; Carnitine; Cholesterol; Diabetes Mellitus, Type 2; Dietary Fiber; Disease Models, Animal; Fatty Acids, Nonesterified; Fatty Liver; Flour; Glucose Tolerance Test; Hordeum; Insulin; Insulin Resistance; Intestine, Small; Ketones; Liver; Obesity; Organ Size; Postprandial Period; Rats; Rats, Zucker; Thiobarbituric Acid Reactive Substances; Triglycerides

2013
Similarities in serum acylcarnitine patterns in type 1 and type 2 diabetes mellitus and in metabolic syndrome.
    Annals of nutrition & metabolism, 2013, Volume: 62, Issue:1

    Topics: Adult; Aged; Body Mass Index; Carnitine; Case-Control Studies; Diabetes Mellitus, Type 1; Diabetes Mellitus, Type 2; Fasting; Female; Humans; Male; Metabolic Syndrome; Middle Aged; Spectrometry, Mass, Electrospray Ionization; Tandem Mass Spectrometry; Young Adult

2013
Performance of serum and dried blood spot acylcarnitine profiles for detection of fatty acid β-oxidation disorders in adult patients with rhabdomyolysis.
    Journal of inherited metabolic disease, 2014, Volume: 37, Issue:2

    Topics: Adolescent; Adult; Biopsy; Carnitine; Carnitine O-Palmitoyltransferase; Dried Blood Spot Testing; Fatty Acids; Female; Humans; Lipid Metabolism, Inborn Errors; Male; Middle Aged; Mitochondrial Diseases; Oxidation-Reduction; Retrospective Studies; Rhabdomyolysis; Young Adult

2014
New autosampler allows quicker, easier dried blood spot analysis.
    Bioanalysis, 2012, Volume: 4, Issue:21

    Topics: Automation; Carnitine; Dried Blood Spot Testing; Humans; Reproducibility of Results; Spectrometry, Mass, Electrospray Ionization

2012
Long-term dietary effects on substrate selection and muscle fiber type in very-long-chain acyl-CoA dehydrogenase deficient (VLCAD(-/-)) mice.
    Biochimica et biophysica acta, 2013, Volume: 1832, Issue:4

    Topics: Acyl-CoA Dehydrogenase, Long-Chain; Animals; Carnitine; Diet, Fat-Restricted; Dietary Fats; Energy Metabolism; Lipids; Lipogenesis; Liver; Mice; Mice, Transgenic; Muscle Fibers, Skeletal; Substrate Specificity; Triglycerides

2013
Sodium oxybate intolerance associated with familial serum acylcarnitine elevation.
    Journal of clinical sleep medicine : JCSM : official publication of the American Academy of Sleep Medicine, 2013, Jan-15, Volume: 9, Issue:1

    Topics: Acyl-CoA Dehydrogenase; Carnitine; Child; Female; Humans; Hypnotics and Sedatives; Male; Middle Aged; Narcolepsy; Polymorphism, Single Nucleotide; Sleep Initiation and Maintenance Disorders; Sodium Oxybate

2013
Carnitine palmitoyltransferase 2 and carnitine/acylcarnitine translocase are involved in the mitochondrial synthesis and export of acylcarnitines.
    FASEB journal : official publication of the Federation of American Societies for Experimental Biology, 2013, Volume: 27, Issue:5

    Topics: Acyl-CoA Dehydrogenase; Carnitine; Carnitine Acyltransferases; Carnitine O-Palmitoyltransferase; Gene Knockdown Techniques; Humans; Membrane Transport Proteins; Mitochondria; Mitochondrial Diseases; Organic Cation Transport Proteins; Solute Carrier Family 22 Member 5

2013
Nutritional and metabolic findings in patients with Prader-Willi syndrome diagnosed in early infancy.
    Journal of pediatric endocrinology & metabolism : JPEM, 2012, Volume: 25, Issue:11-12

    Topics: Arginine; Carboxylic Acids; Carnitine; Chromosomes, Human, Pair 15; Diet Therapy; Early Diagnosis; Failure to Thrive; Female; Gene Deletion; Humans; In Situ Hybridization, Fluorescence; Infant; Infant Nutrition Disorders; Infant, Newborn; Male; Prader-Willi Syndrome; Time-to-Treatment

2012
Rapid quantitative analysis of carnitine and acylcarnitines by ultra-high performance-hydrophilic interaction liquid chromatography-tandem mass spectrometry.
    Journal of chromatography. A, 2013, May-31, Volume: 1292

    Topics: Animals; Carnitine; Chromatography, High Pressure Liquid; Humans; Infant, Newborn; Liver; Muscle, Skeletal; Myocardium; Rats; Tandem Mass Spectrometry

2013
Unique acyl-carnitine profiles are potential biomarkers for acquired mitochondrial disease in autism spectrum disorder.
    Translational psychiatry, 2013, Jan-22, Volume: 3

    Topics: Biomarkers; Carnitine; Child; Child Development Disorders, Pervasive; Cohort Studies; DNA, Mitochondrial; Electron Transport; Fatty Acids; Female; Humans; Male; Mitochondrial Diseases; Muscle, Skeletal; Propionates

2013
Metabolomic distinction of microvascular effects of lipoprotein apheresis--a pilot study.
    Atherosclerosis. Supplements, 2013, Volume: 14, Issue:1

    Topics: Adult; Aged; Biomarkers; Blood Component Removal; Carnitine; Chi-Square Distribution; Decision Trees; Female; Humans; Hyperlipidemias; Lipoproteins; Lysophosphatidylcholines; Male; Metabolomics; Methionine; Microvessels; Middle Aged; Ornithine; Photic Stimulation; Pilot Projects; Retinal Vessels; Risk Assessment; Risk Factors; ROC Curve; Serine; Spectrometry, Mass, Electrospray Ionization; Tandem Mass Spectrometry; Treatment Outcome

2013
Metabolomic analyses of faeces reveals malabsorption in cirrhotic patients.
    Digestive and liver disease : official journal of the Italian Society of Gastroenterology and the Italian Association for the Study of the Liver, 2013, Volume: 45, Issue:8

    Topics: Adult; Amino Acids, Aromatic; Bile Acids and Salts; Bile Pigments; Carnitine; Case-Control Studies; Chromatography, Liquid; Fatty Acids; Feces; Female; Humans; Lipid Metabolism; Liver Cirrhosis; Lysophosphatidylcholines; Malabsorption Syndromes; Male; Mass Spectrometry; Metabolome; Middle Aged

2013
Amino acid and acylcarnitine profiles in premature neonates: a pilot study.
    Indian journal of pediatrics, 2013, Volume: 80, Issue:9

    Topics: Amino Acids; Carnitine; Dried Blood Spot Testing; Female; Gestational Age; Humans; Infant, Newborn; Infant, Premature, Diseases; Longitudinal Studies; Male; Metabolism, Inborn Errors; Neonatal Screening; Reference Values; Spectrometry, Mass, Electrospray Ionization

2013
Aberrant Schwann cell lipid metabolism linked to mitochondrial deficits leads to axon degeneration and neuropathy.
    Neuron, 2013, Mar-06, Volume: 77, Issue:5

    Topics: Aniline Compounds; Animals; Axons; Blotting, Western; Carnitine; Cells, Cultured; Fatty Acids; Fluorescent Dyes; Ganglia, Spinal; Lipid Metabolism; Mice; Mice, Knockout; Mice, Transgenic; Microarray Analysis; Mitochondrial Encephalomyopathies; Nerve Degeneration; Peripheral Nervous System Diseases; Protein Serine-Threonine Kinases; Schwann Cells; Xanthenes

2013
UPLC-MS/MS analysis of C5-acylcarnitines in dried blood spots.
    Clinica chimica acta; international journal of clinical chemistry, 2013, Jun-05, Volume: 421

    Topics: Amino Acid Metabolism, Inborn Errors; Carnitine; Chromatography, High Pressure Liquid; Diagnosis, Differential; Dried Blood Spot Testing; Humans; Infant, Newborn; Isovaleryl-CoA Dehydrogenase; Limit of Detection; Neonatal Screening; Reproducibility of Results; Tandem Mass Spectrometry

2013
Thymidine kinase 2 deficiency-induced mtDNA depletion in mouse liver leads to defect β-oxidation.
    PloS one, 2013, Volume: 8, Issue:3

    Topics: Adenosine Triphosphate; Animals; Carboxypeptidases; Carnitine; Cholesterol; DNA, Mitochondrial; Fatty Acids, Nonesterified; Gene Expression Regulation; Lipid Metabolism; Liver; Mice; Mice, Knockout; Mitochondria, Liver; Oxidation-Reduction; Thymidine Kinase; Transcriptome

2013
Characterization of Pseudomonas aeruginosa growth on O-acylcarnitines and identification of a short-chain acylcarnitine hydrolase.
    Applied and environmental microbiology, 2013, Volume: 79, Issue:11

    Topics: Blotting, Western; Carboxylic Ester Hydrolases; Carnitine; Computational Biology; DNA Primers; Electrophoresis, Polyacrylamide Gel; Enzyme Induction; Genetic Vectors; Hydrolysis; Polymerase Chain Reaction; Pseudomonas aeruginosa; Type C Phospholipases

2013
Leucine and protein metabolism in obese Zucker rats.
    PloS one, 2013, Volume: 8, Issue:3

    Topics: 3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide); Adipose Tissue; Amino Acids, Branched-Chain; Animals; Biomarkers; Body Weight; Carbon Radioisotopes; Carnitine; Chromatography, Liquid; Creatinine; Leucine; Liver; Male; Metabolomics; Muscle, Skeletal; Obesity; Organ Size; Proteins; Rats; Rats, Zucker; Tandem Mass Spectrometry

2013
Metabolomic profiling reveals a role for caspase-2 in lipoapoptosis.
    The Journal of biological chemistry, 2013, May-17, Volume: 288, Issue:20

    Topics: Aminooxyacetic Acid; Animals; Apoptosis; Carnitine; Caspase 2; Cell Death; Chromatography, Gel; Enzyme Activation; Fatty Acids; Gene Expression Regulation, Enzymologic; HEK293 Cells; Hepatocytes; Humans; Metabolomics; Palmitates; RNA, Small Interfering; Xenopus laevis

2013
Carnitine supplementation attenuates myocardial lipid accumulation in long-chain acyl-CoA dehydrogenase knockout mice.
    Journal of inherited metabolic disease, 2013, Volume: 36, Issue:6

    Topics: Acyl-CoA Dehydrogenase, Long-Chain; Animals; Carnitine; Dietary Supplements; Disease Models, Animal; Drug Evaluation, Preclinical; Heart; Lipid Metabolism; Male; Mice; Mice, Inbred C57BL; Mice, Knockout; Myocardium; Triglycerides

2013
Recurrent myoglobinuria and deranged acylcarnitines due to a mutation in the mtDNA MT-CO2 gene.
    Neurology, 2013, May-14, Volume: 80, Issue:20

    Topics: Adult; Carnitine; DNA, Mitochondrial; Electron Transport Complex IV; Humans; Male; Mutation; Myoglobinuria; Recurrence

2013
Production and release of acylcarnitines by primary myotubes reflect the differences in fasting fat oxidation of the donors.
    The Journal of clinical endocrinology and metabolism, 2013, Volume: 98, Issue:6

    Topics: Acyl-CoA Dehydrogenase; Adult; Carnitine; Fasting; Female; Humans; Lipid Metabolism; Male; Muscle Fibers, Skeletal; Oxidation-Reduction

2013
Differential effects of central ghrelin on fatty acid metabolism in hypothalamic ventral medial and arcuate nuclei.
    Physiology & behavior, 2013, Jun-13, Volume: 118

    Topics: Acyl Coenzyme A; AMP-Activated Protein Kinases; Animals; Arcuate Nucleus of Hypothalamus; Blotting, Western; Carboxy-Lyases; Carnitine; Carnitine O-Palmitoyltransferase; Fatty Acids; Ghrelin; Injections, Intraventricular; Lipid Metabolism; Male; Malonyl Coenzyme A; Rats; Rats, Sprague-Dawley; Real-Time Polymerase Chain Reaction; RNA, Messenger; Ventromedial Hypothalamic Nucleus

2013
Matrix-assisted laser desorption/ionization for simultaneous quantitation of (acyl-)carnitines and organic acids in dried blood spots.
    Rapid communications in mass spectrometry : RCM, 2013, Jul-15, Volume: 27, Issue:13

    Topics: Carnitine; Dried Blood Spot Testing; Female; Humans; Infant, Newborn; Male; Mass Spectrometry; Metabolism, Inborn Errors; Neonatal Screening; Organic Chemicals; Spectrometry, Mass, Matrix-Assisted Laser Desorption-Ionization

2013
Important role of ventromedial hypothalamic carnitine palmitoyltransferase-1a in the control of food intake.
    American journal of physiology. Endocrinology and metabolism, 2013, Aug-01, Volume: 305, Issue:3

    Topics: Acyl Coenzyme A; Animals; Appetite Depressants; Arcuate Nucleus of Hypothalamus; Blotting, Western; Body Weight; Carnitine; Carnitine O-Palmitoyltransferase; Dependovirus; Eating; Enzyme Activation; Epoxy Compounds; Fasting; Genetic Vectors; Hypoglycemic Agents; Injections, Intraventricular; Male; Malonyl Coenzyme A; Rats; Rats, Sprague-Dawley; Ventromedial Hypothalamic Nucleus

2013
Plasma carnitine concentrations after chronic alcohol intoxication.
    Postepy higieny i medycyny doswiadczalnej (Online), 2013, May-31, Volume: 67

    Topics: Adult; Alcoholic Intoxication; Alcoholism; Carnitine; Female; Humans; Male; Middle Aged; Plasma; Reference Values; Vitamin B Deficiency; Young Adult

2013
Measurement of free carnitine and acylcarnitines in plasma by HILIC-ESI-MS/MS without derivatization.
    Journal of chromatography. B, Analytical technologies in the biomedical and life sciences, 2013, Aug-01, Volume: 932

    Topics: Carnitine; Chromatography, Liquid; Humans; Hydrophobic and Hydrophilic Interactions; Isomerism; Sensitivity and Specificity; Spectrometry, Mass, Electrospray Ionization; Tandem Mass Spectrometry

2013
Fish oil and the pan-PPAR agonist tetradecylthioacetic acid affect the amino acid and carnitine metabolism in rats.
    PloS one, 2013, Volume: 8, Issue:6

    Topics: Amino Acids; Ammonium Compounds; Animals; Body Weight; Carnitine; Diet; Feeding Behavior; Fish Oils; Gene Expression Regulation; Liver; Male; Peroxisome Proliferator-Activated Receptors; Rats; Rats, Wistar; Sulfides; Urea

2013
Impaired mitochondrial β-oxidation in patients with chronic hepatitis C: relation with viral load and insulin resistance.
    BMC gastroenterology, 2013, Jul-10, Volume: 13

    Topics: Adult; Aged; Carnitine; Fasting; Fatty Acids; Fatty Liver; Female; Hepatitis C, Chronic; Homeostasis; Humans; Insulin; Insulin Resistance; Ketone Bodies; Male; Middle Aged; Mitochondria; Oxidation-Reduction; Viral Core Proteins; Viral Load; Young Adult

2013
Newborn screening for medium-chain acyl-CoA dehydrogenase deficiency: regional experience and high incidence of carnitine deficiency.
    Orphanet journal of rare diseases, 2013, Jul-10, Volume: 8

    Topics: Acyl-CoA Dehydrogenase; Cardiomyopathies; Carnitine; Dietary Supplements; Female; Genetic Association Studies; Genotype; Homozygote; Humans; Hyperammonemia; Incidence; Infant, Newborn; Lipid Metabolism, Inborn Errors; Male; Muscular Diseases; Neonatal Screening; Phenotype; Prevalence; Spain; Vitamin B Complex

2013
Peroxisomes contribute to the acylcarnitine production when the carnitine shuttle is deficient.
    Biochimica et biophysica acta, 2013, Volume: 1831, Issue:9

    Topics: Carnitine; Carnitine Acyltransferases; Carnitine O-Palmitoyltransferase; Cells, Cultured; Fibroblasts; Fluorescent Antibody Technique; Humans; Lauric Acids; Lipid Metabolism, Inborn Errors; Oxidation-Reduction; Peroxisomes; Skin

2013
Five novel mutations in ARG1 gene in Chinese patients of argininemia.
    Pediatric neurology, 2013, Volume: 49, Issue:2

    Topics: Amino Acids; Arginase; Asian People; Carnitine; Child; Child, Preschool; DNA Mutational Analysis; Female; Gas Chromatography-Mass Spectrometry; Humans; Hyperargininemia; Male; Mutation

2013
Acylcarnitine esters profiling of serum and follicular fluid in patients undergoing in vitro fertilization.
    Reproductive biology and endocrinology : RB&E, 2013, Jul-17, Volume: 11

    Topics: Adult; Carnitine; Case-Control Studies; Embryo Transfer; Esters; Female; Fertilization in Vitro; Follicular Fluid; Humans; Male; Oocytes; Pregnancy; Pregnancy Rate; Spectrometry, Mass, Electrospray Ionization; Sperm Injections, Intracytoplasmic; Young Adult

2013
Elaidate, an 18-carbon trans-monoenoic fatty acid, inhibits β-oxidation in human peripheral blood macrophages.
    Journal of cellular biochemistry, 2014, Volume: 115, Issue:1

    Topics: Carnitine; Cells, Cultured; Fatty Acids; Humans; Macrophages; Oleic Acid; Oleic Acids; Oxidation-Reduction; Plant Oils; Stearates; Tandem Mass Spectrometry

2014
Identification and assay of underivatized urinary acylcarnitines by paper spray tandem mass spectrometry.
    Analytical and bioanalytical chemistry, 2013, Volume: 405, Issue:25

    Topics: Adult; Calibration; Carnitine; Humans; Limit of Detection; Paper; Reproducibility of Results; Tandem Mass Spectrometry; Young Adult

2013
Variation of serum metabolites related to habitual diet: a targeted metabolomic approach in EPIC-Potsdam.
    European journal of clinical nutrition, 2013, Volume: 67, Issue:10

    Topics: Adult; Amino Acids; Carnitine; Chronic Disease; Cross-Sectional Studies; Diabetes Mellitus, Type 2; Diet; Feeding Behavior; Female; Hexoses; Humans; Male; Metabolome; Metabolomics; Middle Aged; Phosphatidylcholines; Risk Factors; Surveys and Questionnaires

2013
Glutathione controls the redox state of the mitochondrial carnitine/acylcarnitine carrier Cys residues by glutathionylation.
    Biochimica et biophysica acta, 2013, Volume: 1830, Issue:11

    Topics: Animals; Biological Transport; Carnitine; Carrier Proteins; Cysteine; Glutaredoxins; Glutathione; Glutathione Disulfide; Mitochondria, Liver; Mutagenesis, Site-Directed; Oxidation-Reduction; Proteolipids; Rats

2013
Biochemical competition makes fatty-acid β-oxidation vulnerable to substrate overload.
    PLoS computational biology, 2013, Volume: 9, Issue:8

    Topics: Animals; Carnitine; Fatty Acids; Female; Kinetics; Liver; Metabolic Networks and Pathways; Mitochondria; Models, Biological; NAD; Obesity; Oxidation-Reduction; Palmitoyl Coenzyme A; Palmitoylcarnitine; Rats; Rats, Wistar; Reproducibility of Results

2013
Circulating acylcarnitines as biomarkers of mitochondrial dysfunction after acetaminophen overdose in mice and humans.
    Archives of toxicology, 2014, Volume: 88, Issue:2

    Topics: Acetaminophen; Acetylcysteine; Animals; Antidotes; Biomarkers; Carnitine; Chemical and Drug Induced Liver Injury; Drug Overdose; Humans; Male; Metabolomics; Mice; Mice, Inbred C57BL; Mitochondria

2014
Biotinidase knockout mice show cellular energy deficit and altered carbon metabolism gene expression similar to that of nutritional biotin deprivation: clues for the pathogenesis in the human inherited disorder.
    Molecular genetics and metabolism, 2013, Volume: 110, Issue:3

    Topics: Animals; Biotin; Biotinidase Deficiency; Blood Glucose; Body Weight; Carbon; Carnitine; Diet; Disease Models, Animal; Energy Metabolism; Gene Expression; Humans; Liver; Mice; Mice, Knockout; RNA, Messenger

2013
Daily non-soy legume consumption reverses vascular impairment due to peripheral artery disease.
    Atherosclerosis, 2013, Volume: 230, Issue:2

    Topics: Aged; Aged, 80 and over; Ankle Brachial Index; Atherosclerosis; Biomarkers; Carnitine; Carotid Stenosis; Cholesterol; Cholesterol, LDL; Cohort Studies; Diabetes Mellitus, Type 2; Diet; Endothelium, Vascular; Fabaceae; Female; Humans; Hyperlipidemias; Hypertension; Inflammation; Isoflavones; Male; Middle Aged; Muscle, Skeletal; Peripheral Arterial Disease; Time Factors

2013
A fish protein hydrolysate alters fatty acid composition in liver and adipose tissue and increases plasma carnitine levels in a mouse model of chronic inflammation.
    Lipids in health and disease, 2013, Oct-07, Volume: 12

    Topics: Adipose Tissue; Animals; Carnitine; Caseins; Chronic Disease; Diet, High-Fat; Fatty Acids, Omega-3; Fatty Acids, Omega-6; Female; Fish Proteins; Fishes; Gene Expression; Humans; Inflammation; Liver; Mice; Mice, Transgenic; Ovary; Proteolysis; Tumor Necrosis Factor-alpha

2013
Race and sex differences in small-molecule metabolites and metabolic hormones in overweight and obese adults.
    Omics : a journal of integrative biology, 2013, Volume: 17, Issue:12

    Topics: Aged; Amino Acids, Branched-Chain; Black or African American; Carnitine; Cross-Sectional Studies; Female; Hormones; Humans; Ketones; Male; Metabolome; Middle Aged; Obesity; Overweight; Principal Component Analysis; Sex Characteristics; White People

2013
Identification of biomarkers for apoptosis in cancer cell lines using metabolomics: tools for individualized medicine.
    Journal of internal medicine, 2013, Volume: 274, Issue:5

    Topics: Alanine; Amino Acids; Apoptosis; Biomarkers; Carnitine; Caspase 3; Caspase 7; Cell Line, Tumor; Etoposide; Flow Injection Analysis; Fluorouracil; Glutamic Acid; HEK293 Cells; Hep G2 Cells; Humans; Metabolomics; Precision Medicine

2013
Separation and identification of underivatized plasma acylcarnitine isomers using liquid chromatography-tandem mass spectrometry for the differential diagnosis of organic acidemias and fatty acid oxidation defects.
    Journal of chromatography. A, 2013, Dec-06, Volume: 1319

    Topics: Carnitine; Chromatography, High Pressure Liquid; Diagnosis, Differential; Fatty Acids; Humans; Isomerism; Metabolism, Inborn Errors; Oxidation-Reduction; Reference Standards; Reproducibility of Results; Tandem Mass Spectrometry

2013
Treatable childhood neuronopathy caused by mutations in riboflavin transporter RFVT2.
    Brain : a journal of neurology, 2014, Volume: 137, Issue:Pt 1

    Topics: Adolescent; Brain; Bulbar Palsy, Progressive; Carnitine; Child; Child, Preschool; Exome; Female; Genotype; Hearing Loss, Sensorineural; Humans; Image Processing, Computer-Assisted; Magnetic Resonance Imaging; Male; Microarray Analysis; Motor Neuron Disease; Mutation; Neurologic Examination; Pedigree; Receptors, G-Protein-Coupled; Riboflavin; RNA; Sequence Analysis, DNA; Sural Nerve; Vitamins; Young Adult

2014
Kinetics of carnitine palmitoyltransferase I (CPT I) in Chinese sucker (Myxocyprinus asiaticus) change with its development.
    Lipids, 2014, Volume: 49, Issue:2

    Topics: Animals; Carnitine; Carnitine O-Palmitoyltransferase; Cypriniformes; Fish Proteins; Kinetics; Liver; Muscle, Skeletal

2014
Effect of Allium macrostemon on a rat model of depression studied by using plasma lipid and acylcarnitine profiles from liquid chromatography/mass spectrometry.
    Journal of pharmaceutical and biomedical analysis, 2014, Volume: 89

    Topics: Allium; Animals; Antidepressive Agents; Carnitine; Chromatography, High Pressure Liquid; Depression; Disease Models, Animal; Lipids; Male; Mass Spectrometry; Medicine, Chinese Traditional; Metabolome; Principal Component Analysis; Rats; Rats, Wistar

2014
A plasma long-chain acylcarnitine predicts cardiovascular mortality in incident dialysis patients.
    Journal of the American Heart Association, 2013, Dec-05, Volume: 2, Issue:6

    Topics: Aged; Aged, 80 and over; Biomarkers; Cardiovascular Diseases; Carnitine; Case-Control Studies; Chi-Square Distribution; Chromatography, Liquid; Female; Humans; Kidney Failure, Chronic; Logistic Models; Male; Mass Spectrometry; Metabolomics; Middle Aged; Multivariate Analysis; Odds Ratio; Prospective Studies; Renal Dialysis; Risk Factors; Time Factors; Treatment Outcome; Uremia

2013
Serum metabolic profiles of pregnant women with burdened obstetrical history.
    Bulletin of experimental biology and medicine, 2013, Volume: 156, Issue:1

    Topics: Abortion, Spontaneous; Amino Acids; Biomarkers; Carnitine; Case-Control Studies; Female; Hexoses; Humans; Phosphatidylcholines; Pregnancy; Sphingomyelins

2013
Effect of carnitine, acetyl-, and propionylcarnitine supplementation on the body carnitine pool, skeletal muscle composition, and physical performance in mice.
    European journal of nutrition, 2014, Volume: 53, Issue:6

    Topics: Acetylcarnitine; Administration, Oral; Animals; Biological Availability; Biomarkers; Carnitine; Dietary Supplements; Energy Metabolism; Liver; Male; Mice; Mice, Inbred C57BL; Muscle, Skeletal; Oxidative Stress; Oxygen Consumption; Physical Conditioning, Animal

2014
Changes in mitochondrial carriers exhibit stress-specific signatures in INS-1Eβ-cells exposed to glucose versus fatty acids.
    PloS one, 2013, Volume: 8, Issue:12

    Topics: Animals; Apoptosis; Biological Transport; Carnitine; Cell Line; Cell Survival; DNA; Electron Transport; Energy Metabolism; Fatty Acids; Gene Expression Profiling; Glucose; Insulin; Lipid Metabolism; Mitochondria; Mitochondrial Proteins; Oxidative Phosphorylation; Protein Subunits; Proteome; Rats; Stress, Physiological; Transcription Factors; Transcriptome

2013
Serum levels of acylcarnitines are altered in prediabetic conditions.
    PloS one, 2013, Volume: 8, Issue:12

    Topics: Adult; Aged; Blood Glucose; Body Composition; Carnitine; Diabetes Mellitus, Type 2; Female; Glucose Intolerance; Glucose Tolerance Test; Humans; Male; Middle Aged; Prediabetic State

2013
Obesity and lipid stress inhibit carnitine acetyltransferase activity.
    Journal of lipid research, 2014, Volume: 55, Issue:4

    Topics: Acetyl Coenzyme A; Animals; Carnitine; Carnitine O-Acetyltransferase; Carnitine O-Palmitoyltransferase; Cells, Cultured; Diabetes Mellitus; Humans; Lipid Metabolism; Male; Muscle Fibers, Skeletal; Obesity; Pyruvate Dehydrogenase Complex; Rats, Wistar; Rats, Zucker

2014
Identification of the plasma metabolomics as early diagnostic markers between biliary atresia and neonatal hepatitis syndrome.
    PloS one, 2014, Volume: 9, Issue:1

    Topics: Amino Acids; Biliary Atresia; Biomarkers; Carnitine; Chromatography, Liquid; Demography; Discriminant Analysis; Dried Blood Spot Testing; Early Diagnosis; Female; Hepatitis; Humans; Infant, Newborn; Infant, Newborn, Diseases; Least-Squares Analysis; Male; Mass Spectrometry; Metabolomics; Syndrome

2014
Early mitochondrial dysfunction in glycolytic muscle, but not oxidative muscle, of the fructose-fed insulin-resistant rat.
    American journal of physiology. Endocrinology and metabolism, 2014, Volume: 306, Issue:6

    Topics: Aconitate Hydratase; Animals; Carnitine; Diabetes Mellitus, Type 2; Dietary Carbohydrates; Disease Progression; Energy Metabolism; Fatty Acids; Fructose; Glutamic Acid; Glycolysis; Insulin Resistance; Male; Mitochondria, Muscle; Muscle, Skeletal; Oxidative Phosphorylation; Prediabetic State; Pyruvate Dehydrogenase Complex; Pyruvic Acid; Rats; Rats, Sprague-Dawley; Reactive Oxygen Species

2014
Glutaric aciduria type II presenting as myopathy and rhabdomyolysis in a teenager.
    Journal of child neurology, 2015, Volume: 30, Issue:1

    Topics: Adolescent; Carnitine; Humans; Male; Multiple Acyl Coenzyme A Dehydrogenase Deficiency; Muscular Diseases; Rhabdomyolysis

2015
Metabolomics signature improves the prediction of cardiovascular events in elderly subjects.
    Atherosclerosis, 2014, Volume: 232, Issue:2

    Topics: Aged; Aged, 80 and over; Aging; Biomarkers; Cardiovascular Diseases; Carnitine; Cellular Senescence; Female; Follow-Up Studies; Humans; Male; Metabolic Networks and Pathways; Metabolomics; Myocardial Infarction; Principal Component Analysis; Proportional Hazards Models; Reproducibility of Results; Stroke

2014
Impact of sleep-disordered breathing on myocardial damage and metabolism in patients with chronic heart failure.
    Heart and vessels, 2015, Volume: 30, Issue:3

    Topics: Adult; Aged; Biomarkers; Carnitine; Chronic Disease; Female; Heart Failure; Humans; Male; Middle Aged; Myocardium; Polysomnography; Predictive Value of Tests; Risk Factors; Severity of Illness Index; Sleep Apnea Syndromes; Troponin T; Up-Regulation

2015
The mechanism of Intralipid®-mediated cardioprotection complex IV inhibition by the active metabolite, palmitoylcarnitine, generates reactive oxygen species and activates reperfusion injury salvage kinases.
    PloS one, 2014, Volume: 9, Issue:1

    Topics: Animals; Cardiotonic Agents; Carnitine; Electron Transport Complex IV; Emulsions; Heart; Male; MAP Kinase Signaling System; Mitochondria, Heart; Myocardial Reperfusion Injury; Palmitoylcarnitine; Phospholipids; Proto-Oncogene Proteins c-akt; Rats; Rats, Sprague-Dawley; Reactive Oxygen Species; Soybean Oil; Ventricular Function, Left

2014
Infants suspected to have very-long chain acyl-CoA dehydrogenase deficiency from newborn screening.
    Molecular genetics and metabolism, 2014, Volume: 111, Issue:4

    Topics: Acyl-CoA Dehydrogenase, Long-Chain; Carnitine; Congenital Bone Marrow Failure Syndromes; Demography; DNA Mutational Analysis; Fatty Acids; Female; Genotype; Humans; Infant; Infant, Newborn; Lipid Metabolism, Inborn Errors; Male; Mitochondrial Diseases; Muscular Diseases; Neonatal Screening; Phenotype; Reproducibility of Results

2014
Targeted liquid chromatography-mass spectrometry analysis of serum acylcarnitines in acetaminophen toxicity in children.
    Biomarkers in medicine, 2014, Volume: 8, Issue:2

    Topics: Acetaminophen; Acetylcysteine; Adolescent; Age Factors; Alanine Transaminase; Biomarkers; Carnitine; Chemical and Drug Induced Liver Injury; Child; Child, Preschool; Chromatography, High Pressure Liquid; Discriminant Analysis; Female; Humans; Least-Squares Analysis; Male; Mass Spectrometry; Metabolomics; Sex Factors

2014
The molecular and metabolic influence of long term agmatine consumption.
    The Journal of biological chemistry, 2014, Apr-04, Volume: 289, Issue:14

    Topics: Agmatine; Animals; Biological Transport, Active; Carnitine; Cyclic AMP; Dietary Fats; Fatty Acids; Gene Expression Regulation; Gluconeogenesis; Humans; Liver; Male; Metabolome; Obesity; Oxidation-Reduction; Peroxisome Proliferator-Activated Receptor Gamma Coactivator 1-alpha; PPAR gamma; Rats; Rats, Sprague-Dawley; Time Factors; Transcription Factors

2014
Short-term stability of amino acids and acylcarnitines in the dried blood spots used to screen newborns for metabolic disorders.
    Journal of medical screening, 2014, Volume: 21, Issue:1

    Topics: Amino Acids; Blood Specimen Collection; Carnitine; Dried Blood Spot Testing; Humans; Infant, Newborn; Neonatal Screening; Reproducibility of Results; Sensitivity and Specificity; Specimen Handling; Tandem Mass Spectrometry; Temperature; Time Factors

2014
Linking diet, physical activity, cardiorespiratory fitness and obesity to serum metabolite networks: findings from a population-based study.
    International journal of obesity (2005), 2014, Volume: 38, Issue:11

    Topics: Amino Acids; Biomarkers; Body Mass Index; Carbohydrates; Carnitine; Coffee; Diet; Energy Metabolism; Exercise; Feeding Behavior; Female; Germany; Humans; Life Style; Male; Metabolome; Middle Aged; Obesity; Phospholipids; Physical Fitness; Population Surveillance; Prospective Studies; Risk Factors; Waist Circumference

2014
Fish oil omega-3 fatty acids partially prevent lipid-induced insulin resistance in human skeletal muscle without limiting acylcarnitine accumulation.
    Clinical science (London, England : 1979), 2014, Volume: 127, Issue:5

    Topics: Adult; Carnitine; Fatty Acids, Omega-3; Fish Oils; Glycogen; Humans; Insulin; Insulin Resistance; Lipids; Male; Muscle, Skeletal; Triglycerides

2014
First experience with a fully automated extraction system for simultaneous on-line direct tandem mass spectrometric analysis of amino acids and (acyl-)carnitines in a newborn screening setting.
    Rapid communications in mass spectrometry : RCM, 2014, Apr-30, Volume: 28, Issue:8

    Topics: Amino Acids; Carnitine; Dried Blood Spot Testing; Equipment Design; Humans; Infant, Newborn; Neonatal Screening; Tandem Mass Spectrometry

2014
Plasma acylcarnitines inadequately reflect tissue acylcarnitine metabolism.
    Biochimica et biophysica acta, 2014, Volume: 1841, Issue:7

    Topics: Adipose Tissue, Brown; Adipose Tissue, White; Algorithms; Animals; Carnitine; Cluster Analysis; Fasting; Fatty Acids; Insulin Resistance; Liver; Mice; Mice, Inbred BALB C; Mice, Inbred C57BL; Muscle, Skeletal; Myocardium; Predictive Value of Tests; Tandem Mass Spectrometry

2014
Acylcarnitines activate proinflammatory signaling pathways.
    American journal of physiology. Endocrinology and metabolism, 2014, Jun-15, Volume: 306, Issue:12

    Topics: Animals; Carnitine; Cell Line, Transformed; Cell Line, Tumor; Cyclooxygenase 2; Cytokines; Diabetes Mellitus, Type 2; Docosahexaenoic Acids; Enzyme Induction; Gene Silencing; Humans; Macrophage Activation; Macrophages; MAP Kinase Signaling System; Mice; Myeloid Differentiation Factor 88; Myristic Acids; Phosphorylation; Protein Processing, Post-Translational; Receptors, Pattern Recognition; Recombinant Proteins; Toll-Like Receptor 2

2014
Limited brain metabolism changes differentiate between the progression and clearance of rabies virus.
    PloS one, 2014, Volume: 9, Issue:4

    Topics: 3-Hydroxybutyric Acid; Adaptive Immunity; Animals; Antiviral Agents; Brain; Carnitine; Corticosterone; Disease Progression; Energy Metabolism; Female; Gene Expression; Host-Pathogen Interactions; Hypothalamo-Hypophyseal System; Mice; Pituitary-Adrenal System; Pyridines; Rabies; Rabies virus; Viral Load; Viral Proteins; Viral Vaccines

2014
Urinary metabolomic study of non-small cell lung carcinoma based on ultra high performance liquid chromatography coupled with quadrupole time-of-flight mass spectrometry.
    Journal of separation science, 2014, Volume: 37, Issue:14

    Topics: Adult; Aged; Carnitine; Chromatography, High Pressure Liquid; Female; Humans; Lung Neoplasms; Male; Mass Spectrometry; Metabolomics; Middle Aged; Small Cell Lung Carcinoma; Urine

2014
Functional analysis of iPSC-derived myocytes from a patient with carnitine palmitoyltransferase II deficiency.
    Biochemical and biophysical research communications, 2014, May-30, Volume: 448, Issue:2

    Topics: Bezafibrate; Carnitine; Carnitine O-Palmitoyltransferase; Cell Differentiation; Cells, Cultured; Fibroblasts; Gene Expression Regulation; Humans; Male; Metabolism, Inborn Errors; Muscle Cells; Palmitoylcarnitine; Pluripotent Stem Cells; Young Adult

2014
Modelling of UPLC behaviour of acylcarnitines by quantitative structure-retention relationships.
    Journal of pharmaceutical and biomedical analysis, 2014, Aug-05, Volume: 96

    Topics: Algorithms; Calibration; Carnitine; Chromatography, High Pressure Liquid; Least-Squares Analysis; Linear Models; Models, Theoretical; Principal Component Analysis; Quantitative Structure-Activity Relationship; Reproducibility of Results

2014
Spectrum analysis of common inherited metabolic diseases in Chinese patients screened and diagnosed by tandem mass spectrometry.
    Journal of clinical laboratory analysis, 2015, Volume: 29, Issue:2

    Topics: Acyl-CoA Dehydrogenase; Adolescent; Adult; Amino Acid Metabolism, Inborn Errors; Amino Acids; Carboxylic Acids; Carnitine; Child; Child, Preschool; China; Fatty Acids; Female; Humans; Infant; Infant, Newborn; Lipid Metabolism, Inborn Errors; Male; Metabolic Diseases; Middle Aged; Oxidation-Reduction; Phenylketonurias; Tandem Mass Spectrometry

2015
Human serum acylcarnitine profiles in different glucose tolerance states.
    Diabetes research and clinical practice, 2014, Volume: 104, Issue:3

    Topics: Biomarkers; Blood Glucose; Carnitine; Chromatography, Liquid; Diabetes Mellitus, Type 2; Fasting; Female; Glucose Intolerance; Glucose Tolerance Test; Humans; Male; Middle Aged; Oxidation-Reduction; Prediabetic State; Spectrometry, Mass, Electrospray Ionization

2014
Metabolic heritability at birth: implications for chronic disease research.
    Human genetics, 2014, Volume: 133, Issue:8

    Topics: Acyl-CoA Dehydrogenase; Acyl-CoA Dehydrogenases; Adult; Carnitine; Chronic Disease; Female; Humans; Infant, Newborn; Lipid Metabolism, Inborn Errors; Male; Metabolism, Inborn Errors; Organic Cation Transport Proteins; Symporters; Tandem Mass Spectrometry

2014
Postanalytical tools improve performance of newborn screening by tandem mass spectrometry.
    Genetics in medicine : official journal of the American College of Medical Genetics, 2014, Volume: 16, Issue:12

    Topics: Amino Acids; California; Carnitine; Computational Biology; False Positive Reactions; Female; Humans; Infant, Newborn; Internet; Male; Minnesota; Neonatal Screening; Predictive Value of Tests; Retrospective Studies; Software; Tandem Mass Spectrometry

2014
Long-chain acylcarnitine content determines the pattern of energy metabolism in cardiac mitochondria.
    Molecular and cellular biochemistry, 2014, Volume: 395, Issue:1-2

    Topics: Animals; Carnitine; Energy Metabolism; Fasting; Fatty Acids; Glucose; Male; Mice; Mice, Inbred ICR; Mitochondria, Heart; Models, Animal; Palmitoylcarnitine; Rats; Rats, Wistar

2014
Development of an assay to simultaneously measure orotic acid, amino acids, and acylcarnitines in dried blood spots.
    Clinica chimica acta; international journal of clinical chemistry, 2014, Sep-25, Volume: 436

    Topics: Amino Acids; Carnitine; Dried Blood Spot Testing; Female; Flow Injection Analysis; Humans; Infant; Infant, Newborn; Male; Orotic Acid; Reproducibility of Results; Tandem Mass Spectrometry; Time Factors; Urea Cycle Disorders, Inborn

2014
[C5-carnitine false positive results in newborn screening: what is the cause?].
    Medicina clinica, 2015, Feb-20, Volume: 144, Issue:4

    Topics: Amino Acid Metabolism, Inborn Errors; Biotransformation; Breast Feeding; Carnitine; Coenzyme A Ligases; Cosmetics; False Positive Reactions; Female; Humans; Infant, Newborn; Isovaleryl-CoA Dehydrogenase; Maternal-Fetal Exchange; Neonatal Screening; Ointments; Pentanoic Acids; Pregnancy; Tandem Mass Spectrometry

2015
Continuous-flow microelectroextraction for enrichment of low abundant compounds.
    Analytical chemistry, 2014, Aug-19, Volume: 86, Issue:16

    Topics: Carnitine; Chromatography, Reverse-Phase; Electrochemical Techniques; Equipment Design; Humans; Laurates; Limit of Detection; Mass Spectrometry; Solid Phase Extraction

2014
The effect of long-term oral L-carnitine administration on insulin sensitivity, glucose disposal, plasma concentrations of leptin and acylcarnitines, and urinary acylcarnitine excretion in warmblood horses.
    The veterinary quarterly, 2014, Volume: 34, Issue:2

    Topics: Animals; Carnitine; Glucose Clamp Technique; Horses; Insulin Resistance; Leptin; Male; Plasma; Radioimmunoassay; Vitamin B Complex

2014
Carnitine/acylcarnitine translocase and carnitine palmitoyltransferase 2 form a complex in the inner mitochondrial membrane.
    Molecular and cellular biochemistry, 2014, Volume: 394, Issue:1-2

    Topics: Animals; Blotting, Western; Carnitine; Carnitine Acyltransferases; Carnitine O-Palmitoyltransferase; Cross-Linking Reagents; Formaldehyde; Immunoprecipitation; Membrane Transport Proteins; Mitochondria, Liver; Mitochondrial Membranes; Models, Molecular; Native Polyacrylamide Gel Electrophoresis; Protein Binding; Protein Conformation; Rats

2014
Gestational age and age at sampling influence metabolic profiles in premature infants.
    Pediatrics, 2014, Volume: 134, Issue:1

    Topics: Age Factors; Amino Acids; Carnitine; Female; Gestational Age; Humans; Infant, Newborn; Infant, Premature; Male; Metabolome

2014
Hepatic metabolite profiles in mice with a suboptimal selenium status.
    The Journal of nutritional biochemistry, 2014, Volume: 25, Issue:9

    Topics: Animals; Carnitine; Chromatography, Liquid; Gas Chromatography-Mass Spectrometry; Glycogen; Lipid Peroxidation; Liver; Male; Membrane Lipids; Mice; Mice, Inbred C57BL; Selenium; Tandem Mass Spectrometry; Triiodothyronine

2014
[Carnitine and acylcarnitine percentiles in very low birth weight premature newborn screening samples].
    Anales de pediatria (Barcelona, Spain : 2003), 2015, Volume: 82, Issue:4

    Topics: Carnitine; Female; Humans; Infant, Newborn; Infant, Premature; Infant, Very Low Birth Weight; Male; Neonatal Screening; Reference Values; Retrospective Studies

2015
Metabolite signatures of exercise training in human skeletal muscle relate to mitochondrial remodelling and cardiometabolic fitness.
    Diabetologia, 2014, Volume: 57, Issue:11

    Topics: Adolescent; Adult; Aged; Amino Acids, Branched-Chain; Carnitine; Exercise; Female; Humans; Male; Metabolomics; Middle Aged; Mitochondria, Muscle; Muscle, Skeletal; Succinic Acid; Young Adult

2014
Increased and early lipolysis in children with long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency during fast.
    Journal of inherited metabolic disease, 2015, Volume: 38, Issue:2

    Topics: 3-Hydroxyacyl CoA Dehydrogenases; Age Factors; Biomarkers; Blood Glucose; Calorimetry, Indirect; Cardiomyopathies; Carnitine; Child; Child, Preschool; Energy Metabolism; Fasting; Female; Glycerol; Humans; Hyperglycemia; Isotope Labeling; Lipid Metabolism, Inborn Errors; Lipolysis; Male; Microdialysis; Mitochondrial Myopathies; Mitochondrial Trifunctional Protein; Nervous System Diseases; Postprandial Period; Rhabdomyolysis; Time Factors

2015
Disorders associated with abnormal acylcarnitine profile among high risk Egyptian children.
    Bratislavske lekarske listy, 2014, Volume: 115, Issue:5

    Topics: Biomarkers; Carnitine; Child, Preschool; Chromatography, High Pressure Liquid; Egypt; Female; Humans; Infant; Infant, Newborn; Male; Metabolism, Inborn Errors; Risk Factors; Spectrometry, Mass, Electrospray Ionization

2014
Evidence that multiple defects in lipid regulation occur before hyperglycemia during the prodrome of type-2 diabetes.
    PloS one, 2014, Volume: 9, Issue:9

    Topics: Adiponectin; Adipose Tissue; Adult; Blood Glucose; Carnitine; Diabetes Mellitus, Type 2; Diabetes, Gestational; Diglycerides; Fasting; Female; Follow-Up Studies; Glucose Tolerance Test; Humans; Hyperglycemia; Insulin; Lipid Metabolism; Metabolome; Phospholipids; Prediabetic State; Pregnancy; Principal Component Analysis

2014
Pioglitazone treatment restores in vivo muscle oxidative capacity in a rat model of diabetes.
    Diabetes, obesity & metabolism, 2015, Volume: 17, Issue:1

    Topics: Animals; Biomarkers; Carnitine; Diabetes Mellitus, Type 2; Hypertriglyceridemia; Hypoglycemic Agents; Hypolipidemic Agents; Lipid Metabolism; Male; Mitochondria, Muscle; Mitochondrial Diseases; Mitochondrial Turnover; Muscle, Skeletal; Obesity; Oxidative Phosphorylation; Oxidative Stress; Pioglitazone; PPAR gamma; Rats, Zucker; Thiazolidinediones

2015
Amelioration of acylcarnitine profile using bezafibrate and riboflavin in a case of adult-onset glutaric acidemia type 2 with novel mutations of the electron transfer flavoprotein dehydrogenase (ETFDH) gene.
    Journal of the neurological sciences, 2014, Nov-15, Volume: 346, Issue:1-2

    Topics: Adult; Bezafibrate; Carnitine; Drug Therapy, Combination; Electron-Transferring Flavoproteins; Humans; Hypolipidemic Agents; Iron-Sulfur Proteins; Multiple Acyl Coenzyme A Dehydrogenase Deficiency; Mutation; Oxidoreductases Acting on CH-NH Group Donors; Riboflavin; Treatment Outcome

2014
The first case in Asia of 2-methyl-3-hydroxybutyryl-CoA dehydrogenase deficiency (HSD10 disease) with atypical presentation.
    Journal of human genetics, 2014, Volume: 59, Issue:11

    Topics: 3-Hydroxyacyl CoA Dehydrogenases; Acetyl-CoA C-Acetyltransferase; Base Sequence; Carnitine; Child; Diagnosis, Differential; DNA Mutational Analysis; Dyskinesias; Fibroblasts; Glycine; Humans; Hydroxybutyrates; Immunoblotting; Lipid Metabolism, Inborn Errors; Male; Mental Retardation, X-Linked; Mitochondria; Models, Molecular; Point Mutation; Protein Structure, Tertiary

2014
Analysis and interpretation of acylcarnitine profiles in dried blood spot and plasma of preterm and full-term newborns.
    Pediatric research, 2015, Volume: 77, Issue:1-1

    Topics: Amino Acids, Branched-Chain; Birth Weight; Bronchopulmonary Dysplasia; Carnitine; Dried Blood Spot Testing; Fatty Acids; Female; Gestational Age; Humans; Infant, Newborn; Infant, Premature; Male; Pulmonary Surfactants; Reference Values; Respiration, Artificial; Respiratory Distress Syndrome, Newborn

2015
Familial very long chain acyl-CoA dehydrogenase deficiency as a cause of neonatal sudden infant death: improved survival by prompt diagnosis.
    American journal of medical genetics. Part A, 2015, Volume: 167A, Issue:1

    Topics: Acyl-CoA Dehydrogenase, Long-Chain; Carnitine; Child; Family; Female; Fibroblasts; Humans; Infant; Infant, Newborn; Lipid Metabolism, Inborn Errors; Lymphocytes; Male; Oxidation-Reduction; Palmitates; Perinatal Death; Siblings; Sudden Infant Death; Survival Analysis

2015
Acylcarnitines: potential implications for skeletal muscle insulin resistance.
    FASEB journal : official publication of the Federation of American Societies for Experimental Biology, 2015, Volume: 29, Issue:1

    Topics: Adult; Animals; Antioxidants; Carnitine; Case-Control Studies; Cell Line; Cells, Cultured; Cytokines; Fatty Acids; Female; Humans; Inflammation; Insulin Resistance; Mice; Middle Aged; Muscle Fibers, Skeletal; Muscle, Skeletal; Obesity; Oxidation-Reduction; Oxidative Stress

2015
Genetic activation of pyruvate dehydrogenase alters oxidative substrate selection to induce skeletal muscle insulin resistance.
    Proceedings of the National Academy of Sciences of the United States of America, 2014, Nov-18, Volume: 111, Issue:46

    Topics: Animals; Carnitine; Citric Acid Cycle; Dietary Fats; Enzyme Activation; Fatty Acids; Glucose; Glycogen; Hyperinsulinism; Insulin Resistance; Isoenzymes; Mice; Mice, Inbred C57BL; Mice, Knockout; Models, Biological; Muscle, Skeletal; Nuclear Magnetic Resonance, Biomolecular; Oxidation-Reduction; Oxidative Stress; Phosphorylation; Protein Kinase C; Protein Kinase C-theta; Protein Processing, Post-Translational; Protein Serine-Threonine Kinases; Pyruvate Dehydrogenase Acetyl-Transferring Kinase; Pyruvate Dehydrogenase Complex; Reactive Oxygen Species; RNA, Messenger; Substrate Specificity

2014
Plasma metabolomic profiles of breast cancer patients after short-term limonene intervention.
    Cancer prevention research (Philadelphia, Pa.), 2015, Volume: 8, Issue:1

    Topics: Amino Acids; Anticarcinogenic Agents; Antineoplastic Agents; Bile Acids and Salts; Breast Neoplasms; Carnitine; Cell Cycle; Collagen; Cyclin D1; Cyclohexenes; Female; Gas Chromatography-Mass Spectrometry; Glucose; Humans; Limonene; Metabolome; Metabolomics; Plasma; Terpenes

2015
Newborn screening for medium chain acyl-CoA dehydrogenase deficiency: performance improvement by monitoring a new ratio.
    Molecular genetics and metabolism, 2014, Volume: 113, Issue:4

    Topics: Acyl-CoA Dehydrogenase; Acyl-CoA Dehydrogenases; Biomarkers; Carnitine; False Positive Reactions; Heterozygote; Humans; Infant; Infant, Newborn; Infant, Very Low Birth Weight; Lipid Metabolism, Inborn Errors; Neonatal Screening; United States

2014
Carnitine-acylcarnitine translocase deficiency: Two neonatal cases with common splicing mutation and in vitro bezafibrate response.
    Brain & development, 2015, Volume: 37, Issue:7

    Topics: Bezafibrate; Carnitine; Carnitine Acyltransferases; Cells, Cultured; Child, Preschool; Fatal Outcome; Female; Fibroblasts; Genes, Lethal; Humans; Hypolipidemic Agents; In Vitro Techniques; Infant; Infant, Newborn; Lipid Metabolism, Inborn Errors; Male; Membrane Transport Proteins; Mitochondrial Diseases; Mutation; Treatment Outcome

2015
Mitochondrial remodeling in mice with cardiomyocyte-specific lipid overload.
    Journal of molecular and cellular cardiology, 2015, Volume: 79

    Topics: Adenosine Triphosphate; Animals; Carnitine; Catalase; Ceramides; Cyclic AMP Response Element-Binding Protein; Diglycerides; Electron Transport Complex II; Fatty Acid Transport Proteins; Gene Expression Regulation; Hydrogen Peroxide; Lipids; Mice; Mitochondria, Heart; Models, Biological; Myocardium; Myocytes, Cardiac; Organ Specificity; Oxygen Consumption; Peroxisome Proliferator-Activated Receptor Gamma Coactivator 1-alpha; Phosphorylation; PPAR alpha; Protein Kinase C; Proto-Oncogene Proteins c-akt; RNA, Messenger; Sphingomyelins; Transcription Factors

2015
Fiber-type-specific sensitivities and phenotypic adaptations to dietary fat overload differentially impact fast- versus slow-twitch muscle contractile function in C57BL/6J mice.
    The Journal of nutritional biochemistry, 2015, Volume: 26, Issue:2

    Topics: Acyl-CoA Dehydrogenase; Allostasis; Animals; Carnitine; Diet, Fat-Restricted; Diet, High-Fat; Electron Transport Chain Complex Proteins; Fatty Acids; Glycogen; Male; Mice, Inbred C57BL; Mitochondria, Muscle; Muscle Contraction; Muscle Fibers, Fast-Twitch; Muscle Fibers, Slow-Twitch; Muscle Relaxation; Obesity; Peroxisome Proliferator-Activated Receptor Gamma Coactivator 1-alpha; Random Allocation; Transcription Factors; Troponin T

2015
In HepG2 cells, coexisting carnitine deficiency masks important indicators of marginal biotin deficiency.
    The Journal of nutrition, 2015, Volume: 145, Issue:1

    Topics: Acyl Coenzyme A; Biomarkers; Biotin; Carnitine; Chromatography, Liquid; Culture Media; False Negative Reactions; Female; Hep G2 Cells; Humans; Pregnancy; Tandem Mass Spectrometry

2015
Sodium butyrate epigenetically modulates high-fat diet-induced skeletal muscle mitochondrial adaptation, obesity and insulin resistance through nucleosome positioning.
    British journal of pharmacology, 2015, Volume: 172, Issue:11

    Topics: Adaptation, Physiological; Adiposity; Animals; Blood Glucose; Body Weight; Butyric Acid; Carnitine; Diet, High-Fat; Eating; Energy Metabolism; Epigenesis, Genetic; Insulin Resistance; Mice; Mice, Inbred C57BL; Mitochondria; Mitochondria, Muscle; Muscle Fibers, Slow-Twitch; Muscle, Skeletal; Nucleosomes; Obesity

2015
An audit of newborn screening procedure: impact on infants presenting clinically before results are available.
    Molecular genetics and metabolism, 2015, Volume: 114, Issue:3

    Topics: Amino Acid Metabolism, Inborn Errors; Australia; Carnitine; Female; Health Personnel; Humans; Infant, Newborn; Lipid Metabolism, Inborn Errors; Male; Medical Audit; Neonatal Screening; Retrospective Studies; Tandem Mass Spectrometry; Time Factors

2015
Carnitine supplementation alleviates lipid metabolism derangements and protects against oxidative stress in non-obese hereditary hypertriglyceridemic rats.
    Applied physiology, nutrition, and metabolism = Physiologie appliquee, nutrition et metabolisme, 2015, Volume: 40, Issue:3

    Topics: Animals; Carnitine; Dietary Supplements; DNA, Mitochondrial; Gene Expression Regulation; Genetic Predisposition to Disease; Homeostasis; Hypertriglyceridemia; Insulin Resistance; Kidney; Lipid Metabolism; Liver; Male; Muscle, Skeletal; Oxidative Stress; Rats

2015
Lipid mediators of inflammation as novel plasma biomarkers to identify patients with bacteremia.
    The Journal of infection, 2015, Volume: 70, Issue:5

    Topics: Adult; Aged; Aged, 80 and over; Bacteremia; Biomarkers; Carnitine; Chromatography, High Pressure Liquid; Fatty Acids; Female; Humans; Inflammation Mediators; Lipids; Male; Metabolomics; Middle Aged; Prognosis; ROC Curve; Sphingolipids

2015
Strategies for correcting very long chain acyl-CoA dehydrogenase deficiency.
    The Journal of biological chemistry, 2015, Apr-17, Volume: 290, Issue:16

    Topics: Acetylcysteine; Acyl-CoA Dehydrogenase; Acyl-CoA Dehydrogenase, Long-Chain; Amino Acid Sequence; Carnitine; Congenital Bone Marrow Failure Syndromes; Cysteine; Dose-Response Relationship, Drug; Fatty Acids; Fibroblasts; Genetic Therapy; Humans; Kinetics; Lipid Metabolism, Inborn Errors; Mitochondrial Diseases; Molecular Sequence Data; Muscular Diseases; Mutation; Oxidation-Reduction; Primary Cell Culture; Skin

2015
[Acute encephalopathy induced by vaccination in an infant with methylmalonic aciduria cblA].
    Zhonghua er ke za zhi = Chinese journal of pediatrics, 2015, Volume: 53, Issue:1

    Topics: Amino Acid Metabolism, Inborn Errors; Brain Diseases; Carnitine; Diet, Protein-Restricted; Hepatitis B Vaccines; Humans; Infant; Male; Methylmalonic Acid; Mutation; Vaccination; Vitamin B Complex; Vomiting

2015
Determination of carnitine and acylcarnitines in human urine by means of microextraction in packed sorbent and hydrophilic interaction chromatography-ultra-high-performance liquid chromatography-tandem mass spectrometry.
    Journal of pharmaceutical and biomedical analysis, 2015, May-10, Volume: 109

    Topics: Biomarkers; Calibration; Carnitine; Chromatography, High Pressure Liquid; Humans; Hydrophobic and Hydrophilic Interactions; Indicators and Reagents; Quality Control; Reference Standards; Reproducibility of Results; Solid Phase Microextraction; Tandem Mass Spectrometry

2015
Urinary F2-isoprostanes and metabolic markers of fat oxidation.
    Oxidative medicine and cellular longevity, 2015, Volume: 2015

    Topics: Carnitine; Chromatography, High Pressure Liquid; Cross-Sectional Studies; Diabetes Mellitus, Type 2; F2-Isoprostanes; Fatty Acids; Female; Humans; Logistic Models; Male; Middle Aged; Odds Ratio; Oxidation-Reduction; Risk Factors; Tandem Mass Spectrometry

2015
A case of sudden death after Japanese encephalitis vaccination.
    Legal medicine (Tokyo, Japan), 2015, Volume: 17, Issue:4

    Topics: Anaphylaxis; Arrhythmias, Cardiac; Autopsy; C-Reactive Protein; Carnitine; Cause of Death; Child; Death, Sudden; Encephalitis, Japanese; Fatal Outcome; Heart Arrest; Humans; Japanese Encephalitis Vaccines; Male; Non-alcoholic Fatty Liver Disease; Psychotropic Drugs; Tandem Mass Spectrometry; Uncertainty

2015
Multiple acyl-CoA dehydrogenation deficiency as decreased acyl-carnitine profile in serum.
    Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology, 2015, Volume: 36, Issue:6

    Topics: Acyl Coenzyme A; Carnitine; DNA Mutational Analysis; Electron-Transferring Flavoproteins; Humans; Iron-Sulfur Proteins; Male; Multiple Acyl Coenzyme A Dehydrogenase Deficiency; Muscle, Skeletal; Mutation; Oxidoreductases Acting on CH-NH Group Donors; Young Adult

2015
Acylcarnitine ester utilization by the hindlimb of warmblood horses at rest and following low intensity exercise and carnitine supplementation.
    The veterinary quarterly, 2015, Volume: 35, Issue:2

    Topics: Animals; Carnitine; Dietary Supplements; Female; Hindlimb; Horses; Linear Models; Mass Spectrometry; Motor Activity; Rest

2015
A heterozygous missense mutation in adolescent-onset very long-chain acyl-CoA dehydrogenase deficiency with exercise-induced rhabdomyolysis.
    The Tohoku journal of experimental medicine, 2015, Volume: 235, Issue:4

    Topics: Acyl-CoA Dehydrogenase, Long-Chain; Adolescent; Amino Acid Sequence; Base Sequence; Carnitine; Congenital Bone Marrow Failure Syndromes; Exercise; Heterozygote; Humans; Infant, Newborn; Lipid Metabolism, Inborn Errors; Male; Mitochondrial Diseases; Models, Molecular; Molecular Sequence Data; Muscular Diseases; Mutation, Missense; Protein Structure, Tertiary; Rhabdomyolysis; Sequence Alignment

2015
Long-chain acylcarnitines activate cell stress and myokine release in C2C12 myotubes: calcium-dependent and -independent effects.
    American journal of physiology. Endocrinology and metabolism, 2015, Jun-01, Volume: 308, Issue:11

    Topics: Animals; Calcium; Carnitine; Cell Death; Cell Line; Dose-Response Relationship, Drug; Endoplasmic Reticulum Stress; Interleukin-6; MAP Kinase Signaling System; Mice; Muscle Fibers, Skeletal; Stress, Physiological; Structure-Activity Relationship

2015
Metabolomic analysis reveals altered skeletal muscle amino acid and fatty acid handling in obese humans.
    Obesity (Silver Spring, Md.), 2015, Volume: 23, Issue:5

    Topics: Adult; Amino Acids; Carnitine; Diet, High-Fat; Energy Metabolism; Fatty Acids; Humans; Lipid Metabolism; Male; Metabolomics; Muscle, Skeletal; Obesity; Oxidation-Reduction; Postprandial Period

2015
Medium-chain dicarboxylic acylcarnitines as markers of n-3 PUFA-induced peroxisomal oxidation of fatty acids.
    Molecular nutrition & food research, 2015, Volume: 59, Issue:8

    Topics: Animals; Biomarkers; Carnitine; Diet, High-Fat; Dietary Fats; Dietary Fats, Unsaturated; Fatty Acids, Omega-3; Fish Oils; Gene Expression Regulation; Glucose Intolerance; Hyperlipidemias; Liver; Male; Mice, Inbred C57BL; Molecular Weight; Overweight; Oxidation-Reduction; Peroxisome Proliferators; Peroxisomes; Pyrimidines

2015
Altered Monoamine and Acylcarnitine Metabolites in HIV-Positive and HIV-Negative Subjects With Depression.
    Journal of acquired immune deficiency syndromes (1999), 2015, May-01, Volume: 69, Issue:1

    Topics: Adult; Biogenic Monoamines; Carnitine; Cohort Studies; Depression; Female; HIV Infections; Humans; Male; Metabolome; Middle Aged; Plasma

2015
Dynamic biological changes in metabolic disease biomarkers in childhood and adolescence: A CALIPER study of healthy community children.
    Clinical biochemistry, 2015, Volume: 48, Issue:13-14

    Topics: Adolescent; Amino Acids; Biomarkers; Carnitine; Child; Child, Preschool; Cohort Studies; Female; Health; Humans; Infant, Newborn; Male; Metabolism, Inborn Errors; Reference Values; Residence Characteristics; Young Adult

2015
Neonatal Intrahepatic Cholestasis Caused by Citrin Deficiency Differentiated from Biliary Atresia.
    European journal of pediatric surgery : official journal of Austrian Association of Pediatric Surgery ... [et al] = Zeitschrift fur Kinderchirurgie, 2016, Volume: 26, Issue:3

    Topics: Amino Acids; Analysis of Variance; Biliary Atresia; Bilirubin; Calcium-Binding Proteins; Carnitine; Case-Control Studies; Cholestasis, Intrahepatic; Hexoses; Humans; Infant; Infant, Newborn; Organic Anion Transporters; Retrospective Studies; Sensitivity and Specificity; Tandem Mass Spectrometry

2016
Methyl-γ-butyrobetaine decreases levels of acylcarnitines and attenuates the development of atherosclerosis.
    Vascular pharmacology, 2015, Volume: 72

    Topics: Animals; Aorta; Apolipoproteins E; Atherosclerosis; Betaine; Carnitine; Disease Progression; Female; Male; Mice

2015
Transorgan fluxes in a porcine model reveal a central role for liver in acylcarnitine metabolism.
    American journal of physiology. Endocrinology and metabolism, 2015, Aug-01, Volume: 309, Issue:3

    Topics: Acetylcarnitine; Animals; Carnitine; Catheters, Indwelling; Crosses, Genetic; Female; Intestinal Mucosa; Intestines; Kidney; Lipid Metabolism; Liver; Models, Biological; Olive Oil; Organ Specificity; Palmitoylcarnitine; Plant Oils; Postprandial Period; Sus scrofa

2015
Inherited metabolic disorders in Turkish patients with autism spectrum disorders.
    Autism research : official journal of the International Society for Autism Research, 2016, Volume: 9, Issue:2

    Topics: Adolescent; Adult; Amino Acids; Ammonia; Autism Spectrum Disorder; Blood Gas Analysis; Carnitine; Child; Child, Preschool; Comorbidity; Female; Glycosaminoglycans; Homocysteine; Humans; Infant; Lactic Acid; Male; Metabolism, Inborn Errors; Prevalence; Retrospective Studies; Tandem Mass Spectrometry; Turkey; Young Adult

2016
Metabolism alteration in follicular niche: The nexus among intermediary metabolism, mitochondrial function, and classic polycystic ovary syndrome.
    Free radical biology & medicine, 2015, Volume: 86

    Topics: Adult; Amino Acids, Branched-Chain; Carnitine; Case-Control Studies; Female; Glycolysis; Humans; Ketone Bodies; Mitochondria; Mitophagy; Organelle Biogenesis; Ovarian Follicle; Oxidation-Reduction; Oxidative Stress; Polycystic Ovary Syndrome; Pregnancy; Pregnancy Rate; Reproductive Techniques, Assisted; Treatment Outcome

2015
Female and male human babies have distinct blood metabolomic patterns.
    Molecular bioSystems, 2015, Volume: 11, Issue:9

    Topics: Age Factors; Amino Acids; Carnitine; Dried Blood Spot Testing; Female; Humans; Infant; Infant, Newborn; Male; Metabolome; Metabolomics; Sex Factors; Tandem Mass Spectrometry

2015
First-Trimester Serum Acylcarnitine Levels to Predict Preeclampsia: A Metabolomics Approach.
    Disease markers, 2015, Volume: 2015

    Topics: Adult; Biomarkers; Carnitine; Case-Control Studies; Female; Humans; Metabolomics; Pre-Eclampsia; Pregnancy; Pregnancy Trimester, First; Tandem Mass Spectrometry

2015
Carnitine Acetyltransferase Mitigates Metabolic Inertia and Muscle Fatigue during Exercise.
    Cell metabolism, 2015, Jul-07, Volume: 22, Issue:1

    Topics: Acetyl Coenzyme A; Animals; Carnitine; Carnitine O-Acetyltransferase; Exercise; Humans; Mice, Inbred C57BL; Muscle Fatigue; Muscles; Physical Conditioning, Animal

2015
Effects of waterborne copper exposure on carnitine composition, kinetics of carnitine palmitoyltransferases I (CPT I) and mRNA levels of CPT I isoforms in yellow catfish Pelteobagrus fulvidraco.
    Chemosphere, 2015, Volume: 139

    Topics: Animals; Carnitine; Carnitine O-Palmitoyltransferase; Catfishes; Copper; Environmental Monitoring; Isoenzymes; Kinetics; Lipid Metabolism; Liver; Muscles; RNA, Messenger; Water Pollutants, Chemical

2015
Acylcarnitines are anticoagulants that inhibit factor Xa and are reduced in venous thrombosis, based on metabolomics data.
    Blood, 2015, Sep-24, Volume: 126, Issue:13

    Topics: Adult; Aged; Aged, 80 and over; Anticoagulants; Carnitine; Case-Control Studies; Factor Xa; Factor Xa Inhibitors; Female; Humans; Male; Metabolomics; Middle Aged; Protein Binding; Protein Structure, Tertiary; Risk Factors; Venous Thrombosis; Warfarin

2015
Undernutrition during pregnancy in mice leads to dysfunctional cardiac muscle respiration in adult offspring.
    Bioscience reports, 2015, Apr-10, Volume: 35, Issue:3

    Topics: Animals; Birth Weight; Carnitine; Energy Metabolism; Female; Heart; Liver; Malnutrition; Mice, Inbred ICR; Mitochondria, Heart; Myocardium; Pregnancy; Prenatal Nutritional Physiological Phenomena

2015
An LC-MS/MS method to quantify acylcarnitine species including isomeric and odd-numbered forms in plasma and tissues.
    Journal of lipid research, 2015, Volume: 56, Issue:10

    Topics: Amino Acids; Animals; Carnitine; Chromatography, Liquid; Diabetes Mellitus, Experimental; Fatty Acids; Isomerism; Liver; Mice; Mice, Inbred C57BL; Oxidation-Reduction; Tandem Mass Spectrometry

2015
Long-chain Acylcarnitines Reduce Lung Function by Inhibiting Pulmonary Surfactant.
    The Journal of biological chemistry, 2015, Sep-25, Volume: 290, Issue:39

    Topics: Acyl-CoA Dehydrogenase, Long-Chain; Animals; Carnitine; Humans; Lung; Lung Injury; Mice; Mice, Knockout; Phospholipids; Pulmonary Surfactants

2015
Choline supplementation restores substrate balance and alleviates complications of Pcyt2 deficiency.
    The Journal of nutritional biochemistry, 2015, Volume: 26, Issue:11

    Topics: Acylation; Adipose Tissue; AMP-Activated Protein Kinases; Animals; Carnitine; Choline; Dietary Supplements; Fatty Acids; Gene Expression Regulation; Liver; Mice, Mutant Strains; Phosphatidylcholines; Phosphatidylserines; RNA Nucleotidyltransferases; Triglycerides; Weight Gain

2015
Validated method for the quantification of free and total carnitine, butyrobetaine, and acylcarnitines in biological samples.
    Analytical chemistry, 2015, Sep-01, Volume: 87, Issue:17

    Topics: Animals; Betaine; Carnitine; Chromatography, High Pressure Liquid; Diabetes Mellitus, Experimental; Humans; Mesylates; Muscle, Skeletal; Rats; Solid Phase Extraction; Spectrometry, Mass, Electrospray Ionization; Trimethylsilyl Compounds

2015
Carnitine supplementation in high-fat diet-fed rats does not ameliorate lipid-induced skeletal muscle mitochondrial dysfunction in vivo.
    American journal of physiology. Endocrinology and metabolism, 2015, Oct-01, Volume: 309, Issue:7

    Topics: Animals; Carnitine; Diet, High-Fat; Dietary Supplements; Lipid Metabolism; Lipids; Male; Mitochondria, Muscle; Mitochondrial Diseases; Muscle, Skeletal; Rats; Rats, Wistar

2015
Hypoglycin A Content in Blood and Urine Discriminates Horses with Atypical Myopathy from Clinically Normal Horses Grazing on the Same Pasture.
    PloS one, 2015, Volume: 10, Issue:9

    Topics: Acer; Animals; Carnitine; Disease Outbreaks; Horse Diseases; Horses; Hypoglycins; Muscular Diseases; Plant Poisoning; Plants, Toxic; Seeds; Tandem Mass Spectrometry

2015
Recurrent ACADVL molecular findings in individuals with a positive newborn screen for very long chain acyl-coA dehydrogenase (VLCAD) deficiency in the United States.
    Molecular genetics and metabolism, 2015, Volume: 116, Issue:3

    Topics: Acyl-CoA Dehydrogenase, Long-Chain; Alleles; Carnitine; Computer Simulation; Congenital Bone Marrow Failure Syndromes; Exons; Female; Genetic Carrier Screening; Genotype; Humans; Hypoglycemia; Infant, Newborn; Lipid Metabolism, Inborn Errors; Male; Mitochondrial Diseases; Muscular Diseases; Mutation, Missense; Neonatal Screening; Oligonucleotide Array Sequence Analysis; Sequence Analysis, DNA; Tandem Mass Spectrometry; United States

2015
Integration of Genome-Wide SNP Data and Gene-Expression Profiles Reveals Six Novel Loci and Regulatory Mechanisms for Amino Acids and Acylcarnitines in Whole Blood.
    PLoS genetics, 2015, Volume: 11, Issue:9

    Topics: Amino Acids; Carnitine; Cohort Studies; Disease; Gene Expression Profiling; Genome-Wide Association Study; Humans; Polymorphism, Single Nucleotide; Quantitative Trait Loci

2015
Clinical, biochemical and molecular investigation of adult-onset glutaric acidemia type II: Characteristics in comparison with pediatric cases.
    Brain & development, 2016, Volume: 38, Issue:3

    Topics: Adult; Age Factors; Carnitine; Humans; Male; Multiple Acyl Coenzyme A Dehydrogenase Deficiency; Muscle Weakness; Muscular Diseases

2016
Reference values for amino acids and acylcarnitines in peripheral blood in Quarter horses and American Miniature horses.
    Acta veterinaria Scandinavica, 2015, Sep-29, Volume: 57

    Topics: Amino Acids; Animals; Carnitine; Female; Horses; Male; Mexico; Reference Values; Tandem Mass Spectrometry

2015
Elevation of pivaloylcarnitine by sivelestat sodium in two children.
    Molecular genetics and metabolism, 2015, Volume: 116, Issue:3

    Topics: Amino Acid Metabolism, Inborn Errors; Cardiomyopathies; Carnitine; Child, Preschool; Chromatography, Liquid; Female; Glycine; Humans; Hyperammonemia; Infant, Newborn; Isovaleryl-CoA Dehydrogenase; Muscular Diseases; Neonatal Screening; Serine Proteinase Inhibitors; Sulfonamides; Tandem Mass Spectrometry

2015
Simultaneous quantitation of hexacosanoyl lysophosphatidylcholine, amino acids, acylcarnitines, and succinylacetone during FIA-ESI-MS/MS analysis of dried blood spot extracts for newborn screening.
    Clinical biochemistry, 2016, Volume: 49, Issue:1-2

    Topics: Calibration; Carnitine; Chromatography, High Pressure Liquid; Flow Injection Analysis; Heptanoates; Humans; Infant, Newborn; Lysophosphatidylcholines; Neonatal Screening; Quality Control; Spectrometry, Mass, Electrospray Ionization; Tandem Mass Spectrometry

2016
Quantitative acylcarnitine determination by UHPLC-MS/MS--Going beyond tandem MS acylcarnitine "profiles".
    Molecular genetics and metabolism, 2015, Volume: 116, Issue:4

    Topics: Acetyl-CoA C-Acyltransferase; Acyl-CoA Dehydrogenase; Amino Acid Metabolism, Inborn Errors; Betaine; Carbon-Carbon Ligases; Carnitine; Chromatography, High Pressure Liquid; Female; Humans; Infant, Newborn; Isomerism; Lipid Metabolism, Inborn Errors; Male; Neonatal Screening; Reproducibility of Results; Sensitivity and Specificity; Tandem Mass Spectrometry; Urea Cycle Disorders, Inborn

2015
Metabolic Dysfunction Is Restricted to the Sciatic Nerve in Experimental Diabetic Neuropathy.
    Diabetes, 2016, Volume: 65, Issue:1

    Topics: Animals; Carnitine; Diabetes Mellitus, Experimental; Diabetic Neuropathies; Disease Models, Animal; Energy Metabolism; Fructose; Ganglia, Spinal; Glucose; Homeostasis; Inositol; Lipid Metabolism; Lumbar Vertebrae; Metabolomics; Mitochondria; Neural Conduction; Oxidative Phosphorylation; Oxidative Stress; Polymers; Rats; Rats, Sprague-Dawley; Sciatic Nerve; Sorbitol; Trigeminal Ganglion

2016
Accurate prediction of gestational age using newborn screening analyte data.
    American journal of obstetrics and gynecology, 2016, Volume: 214, Issue:4

    Topics: 17-alpha-Hydroxyprogesterone; Algorithms; Amino Acids; Biomarkers; Biotinidase; Birth Weight; Carnitine; Cross-Sectional Studies; Fatty Acids; Female; Gestational Age; Humans; Infant, Newborn; Logistic Models; Male; Neonatal Screening; Ontario; Oxidation-Reduction; Pregnancy; Thyrotropin; UTP-Hexose-1-Phosphate Uridylyltransferase

2016
Metabolomic Quantitative Trait Loci (mQTL) Mapping Implicates the Ubiquitin Proteasome System in Cardiovascular Disease Pathogenesis.
    PLoS genetics, 2015, Volume: 11, Issue:11

    Topics: Cardiovascular Diseases; Carnitine; DNA Methylation; Endoplasmic Reticulum Stress; Humans; Metabolomics; Polymorphism, Single Nucleotide; Proteasome Endopeptidase Complex; Quantitative Trait Loci; Risk Factors; Ubiquitin

2015
Anaplerotic treatment of long-chain fat oxidation disorders with triheptanoin: Review of 15 years Experience.
    Molecular genetics and metabolism, 2015, Volume: 116, Issue:4

    Topics: Administration, Oral; Adolescent; Adult; Carnitine; Child; Child, Preschool; Citric Acid; Fatty Acids; Female; Humans; Infant; Infant, Newborn; Lactic Acid; Lipid Metabolism, Inborn Errors; Malates; Male; Middle Aged; Mitochondrial Diseases; Oxidation-Reduction; Succinic Acid; Survival Analysis; Treatment Outcome; Triglycerides

2015
Acylcarnitine Profiles in HIV-Exposed, Uninfected Neonates in the United States.
    AIDS research and human retroviruses, 2016, Volume: 32, Issue:4

    Topics: Adult; Anti-HIV Agents; Carnitine; Child Development; Child, Preschool; Cohort Studies; Female; HIV Infections; Humans; Infant; Infant, Newborn; Male; Maternal Exposure; Pregnancy; Pregnancy Complications, Infectious; United States; Young Adult

2016
MK2 Deletion in Mice Prevents Diabetes-Induced Perturbations in Lipid Metabolism and Cardiac Dysfunction.
    Diabetes, 2016, Volume: 65, Issue:2

    Topics: Animals; Carnitine; Diabetes Mellitus, Experimental; Diabetic Cardiomyopathies; Fatty Acids, Nonesterified; Gene Deletion; Hyperglycemia; Insulin; Insulin Resistance; Intracellular Signaling Peptides and Proteins; Ketone Bodies; Lipid Metabolism; Mice; Muscle Contraction; Protein Serine-Threonine Kinases; Streptozocin; Triglycerides

2016
Kinetic and molecular orbital analyses of dicarboxylic acylcarnitine methylesterification show that derivatization may affect the screening of newborns by tandem mass spectrometry.
    Bioorganic & medicinal chemistry letters, 2016, Jan-01, Volume: 26, Issue:1

    Topics: Carnitine; Dicarboxylic Acids; Esterification; Humans; Infant, Newborn; Kinetics; Methylation; Molecular Structure; Neonatal Screening; Quantum Theory; Tandem Mass Spectrometry

2016
Elevations of C14:1 and C14:2 Plasma Acylcarnitines in Fasted Children: A Diagnostic Dilemma.
    The Journal of pediatrics, 2016, Volume: 169

    Topics: Acyl-CoA Dehydrogenase, Long-Chain; Adolescent; Carnitine; Child; Child, Preschool; Congenital Bone Marrow Failure Syndromes; Fasting; Female; Humans; Infant; Lipid Metabolism, Inborn Errors; Male; Mitochondrial Diseases; Muscular Diseases; Retrospective Studies

2016
Quantification of Free Carnitine and Acylcarnitines in Plasma or Serum Using HPLC/MS/MS.
    Methods in molecular biology (Clifton, N.J.), 2016, Volume: 1378

    Topics: Blood Chemical Analysis; Carnitine; Chromatography, High Pressure Liquid; Humans; Reference Standards; Tandem Mass Spectrometry

2016
Decreased acylcarnitine content improves insulin sensitivity in experimental mice models of insulin resistance.
    Pharmacological research, 2016, Volume: 113, Issue:Pt B

    Topics: Animals; Blood Glucose; Carnitine; Diabetes Mellitus; Diabetes Mellitus, Experimental; Diet, High-Fat; Disease Models, Animal; Fatty Acids; gamma-Aminobutyric Acid; Gene Expression; Glucose; Insulin; Insulin Resistance; Lipid Metabolism; Male; Mice; Mice, Inbred C57BL; Muscles; Physical Conditioning, Animal; PPAR alpha; Quaternary Ammonium Compounds; Signal Transduction

2016
Predicting gestational age using neonatal metabolic markers.
    American journal of obstetrics and gynecology, 2016, Volume: 214, Issue:4

    Topics: 17-alpha-Hydroxyprogesterone; Amino Acids; Biomarkers; Birth Weight; Carnitine; Fatty Acids; Female; Gestational Age; Humans; Infant, Newborn; Logistic Models; Male; Neonatal Screening; Pregnancy; Retrospective Studies; Thyrotropin; UTP-Hexose-1-Phosphate Uridylyltransferase

2016
Metabolic changes in serum metabolome in response to a meal.
    European journal of nutrition, 2017, Volume: 56, Issue:2

    Topics: Aged; Amino Acids; Blood Glucose; Bread; Carnitine; Dietary Carbohydrates; Dietary Fats; Dietary Fiber; Dietary Proteins; Energy Metabolism; Fasting; Female; Glycolysis; Humans; Insulin; Ketone Bodies; Leucine; Meals; Metabolome; Middle Aged; Phospholipids; Postmenopause; Postprandial Period; Triticum

2017
Gestational dating by metabolic profile at birth: a California cohort study.
    American journal of obstetrics and gynecology, 2016, Volume: 214, Issue:4

    Topics: 17-alpha-Hydroxyprogesterone; Algorithms; Amino Acids; Biomarkers; California; Carnitine; Cohort Studies; Discriminant Analysis; Female; Gestational Age; Humans; Infant, Newborn; Male; Metabolomics; Multivariate Analysis; Pregnancy; Thyrotropin; Ultrasonography, Prenatal; UTP-Hexose-1-Phosphate Uridylyltransferase

2016
Longitudinal Metabolomic Profiling of Amino Acids and Lipids across Healthy Pregnancy.
    PloS one, 2015, Volume: 10, Issue:12

    Topics: Acetylcarnitine; Adult; Amino Acids; Carnitine; Citric Acid Cycle; Fasting; Fatty Acids; Female; Humans; Lipid Metabolism; Lipids; Longitudinal Studies; Metabolomics; Oxidation-Reduction; Placenta; Pregnancy; Prospective Studies

2015
Simultaneous quantification of cardiovascular disease related metabolic risk factors using liquid chromatography tandem mass spectrometry in human serum.
    Journal of chromatography. B, Analytical technologies in the biomedical and life sciences, 2016, Jan-15, Volume: 1009-1010

    Topics: Adolescent; Adult; Aged; Aged, 80 and over; Amino Acids; Cardiovascular Diseases; Carnitine; Chromatography, Liquid; Female; Humans; Male; Metabolome; Metabolomics; Middle Aged; Risk Factors; Tandem Mass Spectrometry; Young Adult

2016
Acylcarnitines participate in developmental processes associated to lipid metabolism in plants.
    Planta, 2016, Volume: 243, Issue:4

    Topics: Acyl Coenzyme A; Arabidopsis; Arabidopsis Proteins; Brassica napus; Carnitine; Gene Expression Regulation, Plant; Germination; Lipid Metabolism; Plants; Seeds

2016
Lipotoxicity in steatohepatitis occurs despite an increase in tricarboxylic acid cycle activity.
    American journal of physiology. Endocrinology and metabolism, 2016, Apr-01, Volume: 310, Issue:7

    Topics: Animals; Carbon Isotopes; Carnitine; Ceramides; Chromatography, Liquid; Citric Acid Cycle; Dietary Fats; Dietary Sucrose; Diglycerides; Disease Models, Animal; Fatty Acids, Nonesterified; Fructose; Glucose Clamp Technique; Inflammation; Insulin Resistance; Liver; Magnetic Resonance Spectroscopy; Metabolome; Mice; Mitochondria, Liver; Non-alcoholic Fatty Liver Disease; Oxidation-Reduction; Real-Time Polymerase Chain Reaction; RNA, Messenger; Severity of Illness Index; Tandem Mass Spectrometry; Trans Fatty Acids; Transcriptome

2016
Higher plasma levels of lysophosphatidylcholine 18:0 are related to a lower risk of common cancers in a prospective metabolomics study.
    BMC medicine, 2016, Jan-28, Volume: 14

    Topics: Biomarkers, Tumor; Breast Neoplasms; Carnitine; Cohort Studies; Colorectal Neoplasms; Female; Humans; Lipid Metabolism; Lipids; Lysophosphatidylcholines; Male; Middle Aged; Neoplasms; Prospective Studies; Prostatic Neoplasms

2016
Alzheimer's disease-like pathology has transient effects on the brain and blood metabolome.
    Neurobiology of aging, 2016, Volume: 38

    Topics: Aging; Alzheimer Disease; Amino Acids, Branched-Chain; Amino Acids, Essential; Animals; Biogenic Polyamines; Brain; Carnitine; Disease Models, Animal; Male; Metabolome; Mice, Transgenic; Phospholipids; Serotonin

2016
Longitudinal metabolic imaging of hepatocellular carcinoma in transgenic mouse models identifies acylcarnitine as a potential biomarker for early detection.
    Scientific reports, 2016, Feb-02, Volume: 6

    Topics: Animals; Biomarkers; Carcinoma, Hepatocellular; Carnitine; Diffusion Magnetic Resonance Imaging; Disease Models, Animal; Early Detection of Cancer; Gene Expression Profiling; Humans; Liver Neoplasms; Mice; Mice, Transgenic; Molecular Imaging; Tumor Burden

2016
Absolute and Relative Carnitine Deficiency in Patients on Hemodialysis and Peritoneal Dialysis.
    Iranian journal of kidney diseases, 2016, Volume: 10, Issue:1

    Topics: Adolescent; Blood Urea Nitrogen; Carnitine; Child; Female; Humans; Male; Peritoneal Dialysis, Continuous Ambulatory; Renal Dialysis; Renal Insufficiency, Chronic; Young Adult

2016
The Gut Microbiota Modulates Energy Metabolism in the Hibernating Brown Bear Ursus arctos.
    Cell reports, 2016, Feb-23, Volume: 14, Issue:7

    Topics: Actinobacteria; Adaptation, Physiological; Adiposity; Animals; Bacteroidetes; Bile Acids and Salts; Blood Glucose; Carnitine; Cholesterol; Energy Metabolism; Fecal Microbiota Transplantation; Feces; Firmicutes; Gastrointestinal Microbiome; Germ-Free Life; Hibernation; Mice; Principal Component Analysis; Seasons; Triglycerides; Ursidae

2016
Metabolomics Study of Roux-en-Y Gastric Bypass Surgery (RYGB) to Treat Type 2 Diabetes Patients Based on Ultraperformance Liquid Chromatography-Mass Spectrometry.
    Journal of proteome research, 2016, Apr-01, Volume: 15, Issue:4

    Topics: Adult; Amino Acids; Bile Acids and Salts; Bilirubin; Biomarkers; Carnitine; Chromatography, High Pressure Liquid; Diabetes Mellitus, Type 2; Energy Metabolism; Fatty Acids, Nonesterified; Female; Gastric Bypass; Hippurates; Humans; Indican; Insulin Resistance; Male; Mass Spectrometry; Metabolomics; Middle Aged; Obesity, Morbid; Oxidative Stress; Prognosis; Remission Induction; Treatment Outcome; Tryptophan; Weight Loss

2016
Unique plasma metabolomic signatures of individuals with inherited disorders of long-chain fatty acid oxidation.
    Journal of inherited metabolic disease, 2016, Volume: 39, Issue:3

    Topics: Acyl-CoA Dehydrogenase, Long-Chain; Adolescent; Carnitine; Carnitine O-Palmitoyltransferase; Case-Control Studies; Ceramides; Fatty Acids; Female; Humans; Lipid Metabolism; Lipid Metabolism, Inborn Errors; Male; Metabolic Networks and Pathways; Metabolism, Inborn Errors; Oxidation-Reduction; Phosphatidylcholines; Phosphatidylethanolamines; Plasma; Sphingomyelins; Triglycerides

2016
Low levels of short- and medium-chain acylcarnitines in HIV-infected patients.
    European journal of clinical investigation, 2016, Volume: 46, Issue:5

    Topics: Adult; Antiretroviral Therapy, Highly Active; Carnitine; Case-Control Studies; Disease Progression; Female; HIV Infections; Humans; Interferon-gamma; Interferon-gamma Release Tests; Longitudinal Studies; Male; Mycobacterium avium Complex; Mycobacterium avium-intracellulare Infection; Tumor Necrosis Factor-alpha

2016
Muscle and liver-specific alterations in lipid and acylcarnitine metabolism after a single bout of exercise in mice.
    Scientific reports, 2016, Feb-26, Volume: 6

    Topics: Animals; Carnitine; Glycolysis; Lipid Metabolism; Liver; Lysophospholipids; Mice; Muscle, Skeletal; Organ Specificity; Oxidation-Reduction; Physical Conditioning, Animal; Plasmalogens

2016
Altered levels of acylcarnitines, phosphatidylcholines, and sphingomyelins in peritoneal fluid from ovarian endometriosis patients.
    The Journal of steroid biochemistry and molecular biology, 2016, Volume: 159

    Topics: Adult; Area Under Curve; Biomarkers; Carnitine; Case-Control Studies; Endometriosis; Female; Humans; Peritoneum; Phosphatidylcholines; ROC Curve; Sphingomyelins

2016
Metabolomic Profiling of Plasma from Melioidosis Patients Using UHPLC-QTOF MS Reveals Novel Biomarkers for Diagnosis.
    International journal of molecular sciences, 2016, Feb-27, Volume: 17, Issue:3

    Topics: Bacteremia; Biomarkers; Carnitine; Case-Control Studies; Humans; Melioidosis; Metabolome; Phosphatidylcholines; Sphingomyelins

2016
Long-chain acylcarnitines determine ischaemia/reperfusion-induced damage in heart mitochondria.
    The Biochemical journal, 2016, 05-01, Volume: 473, Issue:9

    Topics: Animals; Carnitine; Carnitine O-Palmitoyltransferase; Male; Mitochondria, Heart; Myocardial Reperfusion Injury; Oxidative Phosphorylation; Rats; Rats, Wistar

2016
To treat or not to treat: metabolomics reveals biomarkers for treatment indication in chronic lymphocytic leukaemia patients.
    Oncotarget, 2016, Apr-19, Volume: 7, Issue:16

    Topics: Biomarkers, Tumor; Carnitine; Female; Humans; Leukemia, Lymphocytic, Chronic, B-Cell; Linoleic Acids; Male; Metabolomics; Middle Aged

2016
High incorporation of long-chain fatty acids contributes to the efficient production of acylated ghrelin in ghrelin-producing cells.
    FEBS letters, 2016, Volume: 590, Issue:7

    Topics: Acylation; Acyltransferases; Animals; Carnitine; Cell Line; Coenzyme A Ligases; Enteroendocrine Cells; Enzyme Inhibitors; Fatty Acids, Nonesterified; Gene Expression Profiling; Gene Expression Regulation; Ghrelin; Insulin-Secreting Cells; Lipoylation; Membrane Proteins; Mice, Transgenic; Molecular Weight; Protein Processing, Post-Translational; Recombinant Fusion Proteins; RNA Interference; Triazenes

2016
Significant clinical heterogeneity with similar ETFDH genotype in three Chinese patients with late-onset multiple acyl-CoA dehydrogenase deficiency.
    Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology, 2016, Volume: 37, Issue:7

    Topics: Adolescent; Adult; Aged; Carnitine; Computational Biology; DNA Mutational Analysis; Electron-Transferring Flavoproteins; Genotype; Humans; Iron-Sulfur Proteins; Magnetic Resonance Imaging; Male; Multiple Acyl Coenzyme A Dehydrogenase Deficiency; Muscle Weakness; Muscular Diseases; Mutation; Oxidoreductases Acting on CH-NH Group Donors

2016
Multiple acyl-CoA dehydrogenase deficiency (MADD) as a cause of late-onset treatable metabolic disease.
    Revue neurologique, 2016, Volume: 172, Issue:3

    Topics: Adult; Age of Onset; Biopsy; Carnitine; Electromyography; Electron-Transferring Flavoproteins; Exercise; Female; France; Humans; Iron-Sulfur Proteins; Lipid Metabolism, Inborn Errors; Male; Middle Aged; Multiple Acyl Coenzyme A Dehydrogenase Deficiency; Muscle, Skeletal; Mutation; Neuromuscular Diseases; Oxidation-Reduction; Oxidoreductases Acting on CH-NH Group Donors; Rhabdomyolysis; Young Adult

2016
Systems biology analysis of hepatitis C virus infection reveals the role of copy number increases in regions of chromosome 1q in hepatocellular carcinoma metabolism.
    Molecular bioSystems, 2016, 04-26, Volume: 12, Issue:5

    Topics: Acyl Coenzyme A; Acyltransferases; Carcinoma, Hepatocellular; Carnitine; Chromosomes, Human, Pair 1; Collagen; Diglycerides; DNA Copy Number Variations; Hepacivirus; Hepatitis C; Humans; Keratan Sulfate; Lipid Metabolism; Liver Cirrhosis; Liver Neoplasms; Models, Biological; S-Adenosylmethionine; Systems Biology; Transaminases; Virus Assembly

2016
Clinical Features of Carnitine Deficiency Secondary to Pivalate-Conjugated Antibiotic Therapy.
    The Journal of pediatrics, 2016, Volume: 173

    Topics: Adolescent; Adult; Anti-Bacterial Agents; Brain Diseases; Carnitine; Child; Child, Preschool; Female; Humans; Hypoglycemia; Infant; Male; Retrospective Studies; Tandem Mass Spectrometry; Young Adult

2016
The impact of altered carnitine availability on acylcarnitine metabolism, energy expenditure and glucose tolerance in diet-induced obese mice.
    Biochimica et biophysica acta, 2016, Volume: 1862, Issue:8

    Topics: Animals; Betaine; Carnitine; Dietary Fats; Energy Metabolism; Glucose Intolerance; Insulin Resistance; Liver; Mice; Mice, Obese; Muscle, Skeletal; Obesity

2016
Metabolomic Research on Newborn Infants With Intrauterine Growth Restriction.
    Medicine, 2016, Volume: 95, Issue:17

    Topics: Amino Acids; Carnitine; Female; Fetal Growth Retardation; Gestational Age; Humans; Infant, Newborn; Intensive Care Units, Neonatal; Male; Metabolic Diseases; Metabolomics; Neonatal Screening; Reference Values; Research

2016
C26:0-Carnitine Is a New Biomarker for X-Linked Adrenoleukodystrophy in Mice and Man.
    PloS one, 2016, Volume: 11, Issue:4

    Topics: Acetyltransferases; Adrenoleukodystrophy; Animals; ATP Binding Cassette Transporter, Subfamily D, Member 1; ATP-Binding Cassette Transporters; Biomarkers; Brain; Carnitine; Early Diagnosis; Fatty Acid Elongases; Fatty Acids; Gene Knock-In Techniques; Humans; Lysophosphatidylcholines; Male; Mice; Mice, Inbred C57BL; Mice, Knockout; Oligodendroglia; Spinal Cord

2016
Simultaneous determination of thirteen kinds of amino acid and eight kinds of acylcarnitine in human serum by LC-MS/MS and its application to measure the serum concentration of lung cancer patients.
    Biomedical chromatography : BMC, 2016, Volume: 30, Issue:11

    Topics: Aged; Aged, 80 and over; Amino Acids; Carnitine; Chromatography, High Pressure Liquid; Female; Humans; Limit of Detection; Lung Neoplasms; Male; Middle Aged; Tandem Mass Spectrometry

2016
Influence of moderate maternal nutrition restriction on the fetal baboon metabolome at 0.5 and 0.9 gestation.
    Nutrition, metabolism, and cardiovascular diseases : NMCD, 2016, Volume: 26, Issue:9

    Topics: Adaptation, Physiological; Animals; Biomarkers; Caloric Restriction; Carnitine; Chromatography, Liquid; Disease Models, Animal; Fatty Acids, Nonesterified; Female; Fetus; Gestational Age; Gluconeogenesis; Humans; Liver; Malnutrition; Mass Spectrometry; Maternal Nutritional Physiological Phenomena; Metabolome; Metabolomics; Papio; Phospholipids; Pregnancy

2016
Superior Glucose Tolerance and Metabolomic Profiles, Independent of Adiposity, in HIV-Infected Women Compared With Men on Antiretroviral Therapy.
    Medicine, 2016, Volume: 95, Issue:19

    Topics: Absorptiometry, Photon; Adipose Tissue; Adiposity; Adult; Amino Acids, Branched-Chain; Anti-Retroviral Agents; Blood Glucose; Carnitine; Case-Control Studies; CD4 Lymphocyte Count; Diabetes Mellitus; Fasting; Female; HIV Infections; HIV Seronegativity; Humans; Insulin Resistance; Male; Metabolome; Middle Aged; Obesity; Regression Analysis; Reverse Transcriptase Inhibitors; Risk Factors; Sex Factors; Time Factors

2016
Plasma Acylcarnitines and Amino Acid Levels As an Early Complex Biomarker of Propensity to High-Fat Diet-Induced Obesity in Mice.
    PloS one, 2016, Volume: 11, Issue:5

    Topics: Amino Acids; Analysis of Variance; Animals; Biomarkers; Blood Glucose; Carnitine; Cluster Analysis; Diet, High-Fat; Disease Models, Animal; Female; Glucose Intolerance; Glucose Tolerance Test; Insulin Resistance; Male; Metabolome; Metabolomics; Mice; Obesity; Phenotype; Prognosis; Propensity Score

2016
Metabolic Pathway Signatures Associated with Urinary Metabolite Biomarkers Differentiate Bladder Cancer Patients from Healthy Controls.
    Yonsei medical journal, 2016, Volume: 57, Issue:4

    Topics: Aged; Biomarkers; Carcinoma, Transitional Cell; Carnitine; Case-Control Studies; Female; Humans; Male; Metabolic Networks and Pathways; Middle Aged; Real-Time Polymerase Chain Reaction; RNA, Messenger; Urinary Bladder Neoplasms

2016
Serum Levels of Acyl-Carnitines along the Continuum from Normal to Alzheimer's Dementia.
    PloS one, 2016, Volume: 11, Issue:5

    Topics: Aged; Alzheimer Disease; Analysis of Variance; Biomarkers; Body Mass Index; Calibration; Carnitine; Case-Control Studies; Cognition Disorders; Dementia; Female; Healthy Volunteers; Humans; Male; Middle Aged; Phenotype; Risk

2016
Effect of Exercise and Calorie Restriction on Tissue Acylcarnitines, Tissue Desaturase Indices, and Fat Accumulation in Diet-Induced Obese Rats.
    Scientific reports, 2016, 05-20, Volume: 6

    Topics: Animals; Caloric Restriction; Carnitine; Diet, High-Fat; Disease Models, Animal; Fatty Acid Desaturases; Humans; Insulin Resistance; Liver; Male; Muscle, Skeletal; Obesity; Physical Conditioning, Animal; Rats; Rats, Inbred F344; Treatment Outcome

2016
Outcomes and genotype-phenotype correlations in 52 individuals with VLCAD deficiency diagnosed by NBS and enrolled in the IBEM-IS database.
    Molecular genetics and metabolism, 2016, Volume: 118, Issue:4

    Topics: Acyl-CoA Dehydrogenase, Long-Chain; Adolescent; Carnitine; Child; Child, Preschool; Congenital Bone Marrow Failure Syndromes; Creatine Kinase; Female; Genetic Association Studies; Genotype; Humans; Infant; Infant, Newborn; Lipid Metabolism, Inborn Errors; Male; Mitochondrial Diseases; Muscular Diseases; Mutation; Neonatal Screening; Retrospective Studies

2016
Quantitative analysis of amino acids and acylcarnitines combined with untargeted metabolomics using ultra-high performance liquid chromatography and quadrupole time-of-flight mass spectrometry.
    Journal of chromatography. B, Analytical technologies in the biomedical and life sciences, 2016, Aug-01, Volume: 1027

    Topics: Aged; Amino Acids; Biomarkers; Carnitine; Chromatography, High Pressure Liquid; Female; Humans; Limit of Detection; Male; Metabolomics; Middle Aged; Spectrometry, Mass, Electrospray Ionization

2016
Association between Metabolite Profiles, Metabolic Syndrome and Obesity Status.
    Nutrients, 2016, May-27, Volume: 8, Issue:6

    Topics: Adult; Amino Acids; Body Mass Index; Cardiovascular Diseases; Carnitine; Cholesterol; Cholesterol, HDL; Female; Glycerophospholipids; Humans; Hyperlipidemias; Male; Metabolic Syndrome; Metabolomics; Middle Aged; Obesity; Overweight; Principal Component Analysis; Quebec; Risk Factors; Triglycerides; Waist Circumference

2016
Medium-chain plasma acylcarnitines, ketone levels, cognition, and gray matter volumes in healthy elderly, mildly cognitively impaired, or Alzheimer's disease subjects.
    Neurobiology of aging, 2016, Volume: 43

    Topics: Aged; Aging; Alzheimer Disease; Carnitine; Cognition; Cognitive Dysfunction; Cohort Studies; Female; Gray Matter; Humans; Hydroxybutyrates; Ketone Bodies; Male

2016
Hepatic Fatty Acid Oxidation Restrains Systemic Catabolism during Starvation.
    Cell reports, 2016, 06-28, Volume: 16, Issue:1

    Topics: Adipose Tissue; Animals; Blood Glucose; Body Weight; Carnitine; Carnitine O-Palmitoyltransferase; Diet, Ketogenic; Dyslipidemias; Fasting; Fatty Acids; Gene Expression Regulation; Lipogenesis; Liver; Mice, Knockout; Organ Specificity; Oxidation-Reduction; Oxidative Stress; PPAR alpha; Starvation; Triglycerides

2016
Fermentable soluble fibres spare amino acids in healthy dogs fed a low-protein diet.
    BMC veterinary research, 2016, Jun-28, Volume: 12, Issue:1

    Topics: Acetic Acid; Amino Acids; Animals; Carnitine; Cross-Over Studies; Diet; Dietary Fiber; Dogs; Fatty Acids, Volatile; Feces; Propionates; Random Allocation

2016
Untargeted mass spectrometric approach in metabolic healthy offspring of patients with type 2 diabetes reveals medium-chain acylcarnitine as potential biomarker for lipid induced glucose intolerance (LGIT).
    Archives of physiology and biochemistry, 2016, Volume: 122, Issue:5

    Topics: Adolescent; Adult; Aged; Biomarkers; Blood Glucose; Carnitine; Case-Control Studies; Chromatography, Liquid; Diabetes Mellitus, Type 2; Female; Glucose Intolerance; Glucose Tolerance Test; Humans; Lipids; Male; Mass Spectrometry; Metabolome; Middle Aged; Spectrometry, Mass, Matrix-Assisted Laser Desorption-Ionization; Young Adult

2016
Mitochondrial function is altered in horse atypical myopathy.
    Mitochondrion, 2016, Volume: 30

    Topics: Acer; Animal Feed; Animals; Carnitine; Europe; Female; Horse Diseases; Horses; Hypoglycins; Male; Mitochondrial Diseases; Muscles; Muscular Diseases; Oxidative Phosphorylation

2016
Early Prediction of Developing Type 2 Diabetes by Plasma Acylcarnitines: A Population-Based Study.
    Diabetes care, 2016, Volume: 39, Issue:9

    Topics: Aged; Biomarkers; Blood Glucose; Carnitine; China; Chromatography, Liquid; Diabetes Mellitus, Type 2; Fasting; Female; Glycated Hemoglobin; Humans; Insulin; Lipid Metabolism; Logistic Models; Male; Metabolomics; Middle Aged; Mitochondria; Prospective Studies; Risk Factors; ROC Curve; Tandem Mass Spectrometry

2016
2-Ethylhexanoic acid, found in common plasticizers, leads to an artificial increase in C8 acylcarnitine levels in two neonates treated with extracorporeal membrane oxygenation (ECMO).
    Clinica chimica acta; international journal of clinical chemistry, 2016, Oct-01, Volume: 461

    Topics: Caproates; Carnitine; Extracorporeal Membrane Oxygenation; Humans; Infant, Newborn; Male; Plasticizers

2016
Two transgenic mouse models for β-subunit components of succinate-CoA ligase yielding pleiotropic metabolic alterations.
    The Biochemical journal, 2016, 10-15, Volume: 473, Issue:20

    Topics: Alleles; Animals; Blotting, Western; Carnitine; Cells, Cultured; DNA, Mitochondrial; Heterozygote; Humans; In Vitro Techniques; Membrane Potential, Mitochondrial; Mice; Mice, Knockout; Mice, Mutant Strains; Mitochondria; Phosphorylation; RNA, Messenger; Succinate-CoA Ligases

2016
Efficacy of bezafibrate on fibroblasts of glutaric acidemia type II patients evaluated using an in vitro probe acylcarnitine assay.
    Brain & development, 2017, Volume: 39, Issue:1

    Topics: Adolescent; Adult; Age of Onset; Bezafibrate; Carnitine; Cell Survival; Cells, Cultured; Dose-Response Relationship, Drug; Drug Evaluation, Preclinical; Enzyme Activators; Female; Fibroblasts; Humans; Infant; Infant, Newborn; Lipid Regulating Agents; Male; Multiple Acyl Coenzyme A Dehydrogenase Deficiency; Palmitoylcarnitine; Peroxisome Proliferator-Activated Receptors; Skin

2017
Aerobic capacity and hepatic mitochondrial lipid oxidation alters susceptibility for chronic high-fat diet-induced hepatic steatosis.
    American journal of physiology. Endocrinology and metabolism, 2016, 10-01, Volume: 311, Issue:4

    Topics: Aerobiosis; Animals; Carnitine; Citric Acid Cycle; Diet, High-Fat; Disease Susceptibility; Fatty Acids; Lipid Metabolism; Liver; Male; Mitochondria, Liver; Non-alcoholic Fatty Liver Disease; Oxidation-Reduction; Oxidative Stress; Pyruvic Acid; Rats; Triglycerides

2016
Palmitoyl-carnitine production by blood cells associates with the concentration of circulating acyl-carnitines in healthy overweight women.
    Clinical nutrition (Edinburgh, Scotland), 2017, Volume: 36, Issue:5

    Topics: Adult; Aged; Blood Cells; Blood Glucose; Body Mass Index; Carnitine; Diabetes Mellitus, Type 2; Female; Humans; Hyperinsulinism; Insulin; Insulin Resistance; Lipid Metabolism; Middle Aged; Overweight; Oxidation-Reduction; Palmitates; Palmitoylcarnitine

2017
Liver and Muscle Contribute Differently to the Plasma Acylcarnitine Pool During Fasting and Exercise in Humans.
    The Journal of clinical endocrinology and metabolism, 2016, Volume: 101, Issue:12

    Topics: Adult; Carnitine; Exercise; Fasting; Humans; Leg; Liver; Male; Muscle, Skeletal; Young Adult

2016
Increased C3 Acylcarnitine Concentration in a Newborn.
    Clinical chemistry, 2016, Volume: 62, Issue:10

    Topics: Amino Acid Metabolism, Inborn Errors; Carnitine; Female; Humans; Hydroxocobalamin; Infant, Newborn; Mitochondrial Membrane Transport Proteins; Mutation; Neonatal Screening; Treatment Outcome

2016
Plasma acylcarnitines during insulin stimulation in humans are reflective of age-related metabolic dysfunction.
    Biochemical and biophysical research communications, 2016, Oct-28, Volume: 479, Issue:4

    Topics: Acetyl-CoA Carboxylase; Adult; Aged; Aged, 80 and over; Aging; Amino Acids; Carnitine; Female; Glucose; Glucose Clamp Technique; Humans; Insulin; Insulin Resistance; Male; Middle Aged; Muscle, Skeletal; Oxidation-Reduction; Phosphorylation; Young Adult

2016
Plasma acylcarnitine profiling indicates increased fatty acid oxidation relative to tricarboxylic acid cycle capacity in young, healthy low birth weight men.
    Physiological reports, 2016, Volume: 4, Issue:19

    Topics: Adult; Birth Weight; Carnitine; Citric Acid Cycle; Cross-Over Studies; Denmark; Diabetes Mellitus, Type 2; Diet, High-Fat; Dietary Fats; Energy Intake; Fasting; Fatty Acids; Humans; Infant, Low Birth Weight; Insulin; Insulin Resistance; Lipid Metabolism; Male; Mitochondria; Oxidation-Reduction; Randomized Controlled Trials as Topic

2016
Oleate dose-dependently regulates palmitate metabolism and insulin signaling in C2C12 myotubes.
    Biochimica et biophysica acta, 2016, Volume: 1861, Issue:12 Pt A

    Topics: Animals; Carnitine; Cell Line; Ceramides; Diglycerides; Fatty Acids; Insulin; Insulin Resistance; Mice; Muscle Fibers, Skeletal; Muscle, Skeletal; NF-kappa B; Oleic Acid; Oxidation-Reduction; p38 Mitogen-Activated Protein Kinases; Palmitates; Phosphorylation; Proto-Oncogene Proteins c-akt; Signal Transduction; Triglycerides

2016
Acylcarnitines as markers of exercise-associated fuel partitioning, xenometabolism, and potential signals to muscle afferent neurons.
    Experimental physiology, 2017, 01-01, Volume: 102, Issue:1

    Topics: Adenosine Triphosphate; Adult; Amino Acids, Branched-Chain; Biomarkers; Carnitine; Citric Acid Cycle; Diabetes Mellitus, Type 2; Exercise; Fatty Acids; Female; Humans; Insulin Resistance; Middle Aged; Mitochondria, Muscle; Muscle, Skeletal; Neurons, Afferent; Obesity; Oxidation-Reduction; Oxidative Phosphorylation; Oxygen Consumption; Weight Loss

2017
[An analysis of clinical characteristics and gene mutation in two patients with medium- and short-chain acyl-CoA dehydrogenase deficiency].
    Zhongguo dang dai er ke za zhi = Chinese journal of contemporary pediatrics, 2016, Volume: 18, Issue:10

    Topics: Acyl-CoA Dehydrogenase; Carnitine; Female; Humans; Infant; Infant, Newborn; Lipid Metabolism, Inborn Errors; Male; Mutation; Urea

2016
Metabolite and Lipid Profiling of Biobank Plasma Samples Collected Prior to Onset of Rheumatoid Arthritis.
    PloS one, 2016, Volume: 11, Issue:10

    Topics: Adult; Area Under Curve; Arthritis, Rheumatoid; Biological Specimen Banks; Biomarkers; Body Mass Index; Carnitine; Chromatography, High Pressure Liquid; Discriminant Analysis; Fatty Acids; Female; Humans; Least-Squares Analysis; Lipids; Lysophosphatidylcholines; Metabolomics; Middle Aged; Principal Component Analysis; ROC Curve; Tryptophan

2016
Novel Molecular Interactions of Acylcarnitines and Fatty Acids with Myoglobin.
    The Journal of biological chemistry, 2016, Nov-25, Volume: 291, Issue:48

    Topics: Animals; Carnitine; Fatty Acids; Horses; Models, Chemical; Myoglobin

2016
Cardiovascular Risk Factors Associated With Blood Metabolite Concentrations and Their Alterations During a 4-Year Period in a Population-Based Cohort.
    Circulation. Cardiovascular genetics, 2016, Volume: 9, Issue:6

    Topics: Aged; Aged, 80 and over; Alcohol Drinking; Amino Acids; Biomarkers; Body Mass Index; Cardiovascular Diseases; Carnitine; Cross-Sectional Studies; Female; Germany; Hexoses; Humans; Linear Models; Lipids; Longitudinal Studies; Male; Metabolomics; Middle Aged; Multivariate Analysis; Obesity; Risk Assessment; Risk Factors; Sex Factors; Smoking; Time Factors

2016
Acylcarnitine Profiles Reflect Metabolic Vulnerability for Necrotizing Enterocolitis in Newborns Born Premature.
    The Journal of pediatrics, 2017, Volume: 181

    Topics: Biomarkers; California; Carnitine; Cohort Studies; Confidence Intervals; Enterocolitis, Necrotizing; Female; Follow-Up Studies; Gestational Age; Humans; Incidence; Infant, Newborn; Infant, Premature; Intensive Care Units, Neonatal; Male; Multivariate Analysis; Neonatal Screening; Odds Ratio; Reproducibility of Results; Retrospective Studies; Risk Assessment; Vulnerable Populations

2017
Analysis of amino acids and acyl carnitine profiles in low birth weight, preterm, and small for gestational age neonates.
    The journal of maternal-fetal & neonatal medicine : the official journal of the European Association of Perinatal Medicine, the Federation of Asia and Oceania Perinatal Societies, the International Society of Perinatal Obstetricians, 2017, Volume: 30, Issue:22

    Topics: Amino Acids; Carnitine; Dried Blood Spot Testing; Female; Gestational Age; Humans; Infant, Low Birth Weight; Infant, Newborn; Infant, Premature; Infant, Small for Gestational Age; Male; Metabolome; Retrospective Studies

2017
Living on the edge: substrate competition explains loss of robustness in mitochondrial fatty-acid oxidation disorders.
    BMC biology, 2016, 12-07, Volume: 14, Issue:1

    Topics: Acyl-CoA Dehydrogenase; Animals; Carnitine; Computational Biology; Computer Simulation; Disease Models, Animal; Fatty Acids; Humans; Lipid Metabolism; Lipid Metabolism, Inborn Errors; Male; Metabolic Networks and Pathways; Mice; Mice, Inbred C57BL; Mice, Knockout; Mitochondria; Oxidation-Reduction; Proteomics; Substrate Specificity

2016
Study of Carnitine/Acylcarnitine and Amino Acid Profile in Children and Adults With Acute Liver Failure.
    Journal of pediatric gastroenterology and nutrition, 2017, Volume: 64, Issue:6

    Topics: Adolescent; Adult; Amino Acids; Biomarkers; Carnitine; Carnitine O-Palmitoyltransferase; Child; Child, Preschool; Female; Follow-Up Studies; Humans; Hypoglycemia; Infant; Lipid Metabolism, Inborn Errors; Liver Failure, Acute; Liver Transplantation; Male; Patient Acuity; Prospective Studies

2017
Change of the metabolomic profile during short-term mononuclear cell storage.
    Vox sanguinis, 2017, Volume: 112, Issue:2

    Topics: Amines; Amino Acids; Apoptosis; Blood Preservation; Carnitine; CD4-Positive T-Lymphocytes; Chromatography, High Pressure Liquid; Glucose; Humans; Leukapheresis; Leukocytes, Mononuclear; Metabolomics; Phospholipids; Sphingolipids; Tandem Mass Spectrometry; Time Factors

2017
A 61-Year-Old Woman with Muscle Fatigue and Increased Cardiac Troponin.
    Clinical chemistry, 2017, Volume: 63, Issue:1

    Topics: Carnitine; Creatine Kinase; Female; Humans; Lipid Metabolism, Inborn Errors; Middle Aged; Multiple Acyl Coenzyme A Dehydrogenase Deficiency; Muscle Fatigue; Muscle, Skeletal; Muscular Dystrophies; Necrosis; Troponin I; Troponin T

2017
Profiles of Amino Acids and Acylcarnitines Related with Insecticide Exposure in Culex quinquefasciatus (Say).
    PloS one, 2017, Volume: 12, Issue:1

    Topics: Amino Acids; Animals; Carnitine; Culex; Insecticides; Larva; Metabolome; Tandem Mass Spectrometry

2017
Mitochondrial trifunctional protein deficiency: an adult patient with similar progress to Charcot-Marie-Tooth disease.
    Rinsho shinkeigaku = Clinical neurology, 2017, 02-25, Volume: 57, Issue:2

    Topics: Biomarkers; Cardiomyopathies; Carnitine; Charcot-Marie-Tooth Disease; Diagnosis, Differential; Disease Progression; Genetic Testing; Humans; Lipid Metabolism, Inborn Errors; Male; Middle Aged; Mitochondrial Myopathies; Mitochondrial Trifunctional Protein; Mitochondrial Trifunctional Protein, beta Subunit; Mutation; Nervous System Diseases; Peripheral Nervous System Diseases; Recurrence; Rhabdomyolysis

2017
Serum Acylcarnitines and Risk of Cardiovascular Death and Acute Myocardial Infarction in Patients With Stable Angina Pectoris.
    Journal of the American Heart Association, 2017, 02-03, Volume: 6, Issue:2

    Topics: Aged; Angina Pectoris; Biomarkers; Carnitine; Cause of Death; Follow-Up Studies; Heart Arrest; Humans; Middle Aged; Myocardial Infarction; Norway; Prognosis; Prospective Studies; Risk Assessment; Risk Factors; Survival Rate

2017
Specific circulating phospholipids, acylcarnitines, amino acids and biogenic amines are aerobic exercise markers.
    Journal of science and medicine in sport, 2017, Volume: 20, Issue:7

    Topics: Amino Acids; Biogenic Amines; Biomarkers; Carnitine; Chromatography, Liquid; Cross-Sectional Studies; Exercise; Humans; Male; Middle Aged; Phospholipids; Tandem Mass Spectrometry

2017
Impact of a cafeteria diet and daily physical training on the rat serum metabolome.
    PloS one, 2017, Volume: 12, Issue:2

    Topics: Animals; Bile Acids and Salts; C-Reactive Protein; Carnitine; Chromatography, Liquid; Diet; Fatty Acids, Unsaturated; Female; Glycerophospholipids; Leptin; Metabolome; Metabolomics; Physical Conditioning, Animal; Rats, Sprague-Dawley; Tandem Mass Spectrometry; Time Factors; Vitamin A; Vitamin E

2017
Decrease in Long-Chain Acylcarnitine Tissue Content Determines the Duration of and Correlates with the Cardioprotective Effect of Methyl-GBB.
    Basic & clinical pharmacology & toxicology, 2017, Volume: 121, Issue:2

    Topics: Animals; Betaine; Biotransformation; Cardiotonic Agents; Carnitine; Down-Regulation; gamma-Aminobutyric Acid; Half-Life; Heart; Male; Membrane Transport Modulators; Molecular Weight; Myocardial Infarction; Myocardium; Perfusion; Quaternary Ammonium Compounds; Random Allocation; Rats, Wistar; Solute Carrier Family 22 Member 5; Time Factors; Tissue Distribution

2017
Endometriosis is associated with aberrant metabolite profiles in plasma.
    Fertility and sterility, 2017, Volume: 107, Issue:3

    Topics: Adult; Area Under Curve; Belgium; Biomarkers; Carnitine; Case-Control Studies; Chromatography, High Pressure Liquid; Endometriosis; Female; Hospitals, University; Humans; Laparoscopy; Lipids; Metabolomics; Pilot Projects; Predictive Value of Tests; ROC Curve; Spectrometry, Mass, Electrospray Ionization; Tandem Mass Spectrometry

2017
Matrix effect-corrected liquid chromatography/tandem mass-spectrometric method for determining acylcarnitines in human urine.
    Clinica chimica acta; international journal of clinical chemistry, 2017, Volume: 468

    Topics: Adult; Calibration; Carnitine; Child; Chromatography, Liquid; Humans; Reproducibility of Results; Tandem Mass Spectrometry; Urinalysis

2017
Microbiome and metabolome modifying effects of several cardiovascular disease interventions in apo-E
    Microbiome, 2017, 03-13, Volume: 5, Issue:1

    Topics: Acetates; Animals; Apolipoproteins E; Atherosclerosis; Atorvastatin; beta-Glucans; Butyrates; Cardiovascular Diseases; Carnitine; Cholesterol; Cholesterol, Dietary; Diet, High-Fat; Dietary Supplements; Feces; Gastrointestinal Microbiome; Hemiterpenes; Limosilactobacillus reuteri; Male; Metabolome; Mice; Obesity; Pentanoic Acids; Probiotics

2017
Rapid differentiating colorectal cancer and colorectal polyp using dried blood spot mass spectrometry metabolomic approach.
    IUBMB life, 2017, Volume: 69, Issue:5

    Topics: Adult; Aged; Aged, 80 and over; Amino Acids; Carnitine; Colonic Polyps; Colorectal Neoplasms; Dried Blood Spot Testing; Female; Humans; Male; Metabolomics; Middle Aged; Reproducibility of Results

2017
Systems characterization of differential plasma metabolome perturbations following thrombotic and non-thrombotic myocardial infarction.
    Journal of proteomics, 2017, 05-08, Volume: 160

    Topics: Adult; Aged; Blood; Carnitine; Coronary Thrombosis; Female; Gas Chromatography-Mass Spectrometry; Glucocorticoids; Humans; Hypoxia; Male; Metabolome; Metabolomics; Middle Aged; Monoglycerides; Myocardial Infarction; Oxygen Consumption; Pregnenolone; Tandem Mass Spectrometry

2017
Increased urine acylcarnitines in diabetic ApoE-/- mice: Hydroxytetradecadienoylcarnitine (C14:2-OH) reflects diabetic nephropathy in a context of hyperlipidemia.
    Biochemical and biophysical research communications, 2017, 05-20, Volume: 487, Issue:1

    Topics: Animals; Apolipoproteins E; Biomarkers; Carnitine; Diabetic Nephropathies; Hyperlipidemias; Male; Mice; Mice, Knockout; Up-Regulation

2017
Amino acid and acylcarnitine profiles in perinatal asphyxia: a case-control study.
    The journal of maternal-fetal & neonatal medicine : the official journal of the European Association of Perinatal Medicine, the Federation of Asia and Oceania Perinatal Societies, the International Society of Perinatal Obstetricians, 2018, Volume: 31, Issue:11

    Topics: Amino Acids; Asphyxia Neonatorum; Carnitine; Case-Control Studies; Chromatography, Liquid; Female; Humans; Hypoxia-Ischemia, Brain; Infant, Newborn; Male; Mass Spectrometry; Metabolome

2018
Dynamics of L-Carnitine in Plasma and Urine in Patients Undergoing Cisplatin Chemotherapy.
    Chemotherapy, 2017, Volume: 62, Issue:4

    Topics: Antineoplastic Agents; Body Mass Index; Carnitine; Cisplatin; Female; Glomerular Filtration Rate; Humans; Male; Middle Aged; Muscle, Skeletal; Neoplasms

2017
Nitric oxide inhibits the mitochondrial carnitine/acylcarnitine carrier through reversible S-nitrosylation of cysteine 136.
    Biochimica et biophysica acta. Bioenergetics, 2017, Volume: 1858, Issue:7

    Topics: Amino Acid Sequence; Animals; Biological Transport; Carnitine; Carnitine Acyltransferases; Conserved Sequence; Cysteine; Dithioerythritol; Liposomes; Mitochondria; Models, Molecular; Nitric Oxide; Nitric Oxide Donors; Nitrogen; Oxidation-Reduction; Protein Conformation; Protein Processing, Post-Translational; Rats; S-Nitrosoglutathione; Sequence Alignment; Sequence Homology, Amino Acid

2017
Changes in Hepatic TRβ Protein Expression, Lipogenic Gene Expression, and Long-Chain Acylcarnitine Levels During Chronic Hyperthyroidism and Triiodothyronine Withdrawal in a Mouse Model.
    Thyroid : official journal of the American Thyroid Association, 2017, Volume: 27, Issue:6

    Topics: Adipogenesis; Animals; Body Weight; Carnitine; Chronic Disease; Disease Models, Animal; Gene Expression Regulation; Hyperthyroidism; Lipogenesis; Liver; Male; Metabolomics; Mice; Mice, Inbred C57BL; Mice, Knockout; Thyroid Hormone Receptors beta; Thyroid Hormones; Time Factors; Triiodothyronine

2017
Metabolomic profiling of ascending thoracic aortic aneurysms and dissections - Implications for pathophysiology and biomarker discovery.
    PloS one, 2017, Volume: 12, Issue:5

    Topics: Adult; Aged; Amino Acids; Aorta, Thoracic; Aortic Aneurysm, Thoracic; Aortic Dissection; Biomarkers; Carnitine; Case-Control Studies; Ceramides; Female; Hexoses; Humans; Lysophosphatidylcholines; Male; Metabolomics; Middle Aged; Phosphatidylcholines; Sphingomyelins; Young Adult

2017
Developmental regulation and localization of carnitine palmitoyltransferases (CPTs) in rat brain.
    Journal of neurochemistry, 2017, Volume: 142, Issue:3

    Topics: Acyl Coenzyme A; Animals; Brain; Carnitine; Carnitine O-Palmitoyltransferase; Fatty Acids; Liver; Male; Mitochondria; Oleic Acid; Rats, Sprague-Dawley

2017
Sustained NFκB inhibition improves insulin sensitivity but is detrimental to muscle health.
    Aging cell, 2017, Volume: 16, Issue:4

    Topics: Aging; Animals; Blood Glucose; Carnitine; Cell Line; Ceramides; Female; Gene Expression Regulation; Humans; Insulin Resistance; Male; Mice; Mice, Inbred C57BL; Muscle, Skeletal; Muscular Atrophy; Myoblasts; Myostatin; NF-kappa B; NF-KappaB Inhibitor alpha; Sarcopenia

2017
Identification and diagnostic value of phytanoyl- and pristanoyl-carnitine in plasma from patients with peroxisomal disorders.
    Molecular genetics and metabolism, 2017, Volume: 121, Issue:3

    Topics: Carnitine; Cells, Cultured; Diterpenes; Fatty Acids; Humans; Oxidation-Reduction; Peroxisomal Disorders; Phytanic Acid; Refsum Disease

2017
Development of a Data-Independent Targeted Metabolomics Method for Relative Quantification Using Liquid Chromatography Coupled with Tandem Mass Spectrometry.
    Analytical chemistry, 2017, 07-05, Volume: 89, Issue:13

    Topics: Biomarkers; Carnitine; Chromatography, Liquid; Humans; Male; Metabolome; Metabolomics; Middle Aged; Tandem Mass Spectrometry

2017
N-acyl Taurines and Acylcarnitines Cause an Imbalance in Insulin Synthesis and Secretion Provoking β Cell Dysfunction in Type 2 Diabetes.
    Cell metabolism, 2017, Jun-06, Volume: 25, Issue:6

    Topics: Animals; Carnitine; Diabetes Mellitus, Type 2; Humans; Insulin; Insulin Secretion; Insulin-Secreting Cells; Mice; Taurine

2017
[Fat emulsion tolerance in preterm infants of different gestational ages in the early stage after birth].
    Zhongguo dang dai er ke za zhi = Chinese journal of contemporary pediatrics, 2017, Volume: 19, Issue:6

    Topics: Carnitine; Fat Emulsions, Intravenous; Gestational Age; Humans; Infant, Newborn; Infant, Premature

2017
Association between resistance to erythropoiesis-stimulating agents and carnitine profile in patients on maintenance haemodialysis.
    Nephrology (Carlton, Vic.), 2018, Volume: 23, Issue:8

    Topics: Adult; Aged; Anemia; Biomarkers; Carnitine; Chromatography, Liquid; Cross-Sectional Studies; Drug Resistance; Female; Hematinics; Humans; Kidney Failure, Chronic; Male; Middle Aged; Renal Dialysis; Retrospective Studies; Risk Factors; Tandem Mass Spectrometry; Treatment Outcome

2018
Targeted Metabolomics Demonstrates Distinct and Overlapping Maternal Metabolites Associated With BMI, Glucose, and Insulin Sensitivity During Pregnancy Across Four Ancestry Groups.
    Diabetes care, 2017, Volume: 40, Issue:7

    Topics: Amino Acids; Blood Glucose; Body Mass Index; Carnitine; Cohort Studies; Female; Gestational Age; Glucose Tolerance Test; Humans; Hyperglycemia; Insulin Resistance; Metabolomics; Pregnancy; Pregnancy Complications; Pregnancy Outcome; Racial Groups

2017
Early Postnatal Diets Affect the Bioregional Small Intestine Microbiome and Ileal Metabolome in Neonatal Pigs.
    The Journal of nutrition, 2017, Volume: 147, Issue:8

    Topics: Aminoisobutyric Acids; Animals; Animals, Newborn; Bacteria; Carnitine; Diet; Duodenum; Feeding Behavior; Female; Food, Formulated; Gastrointestinal Microbiome; Humans; Ileum; Intestine, Small; Male; Metabolome; Milk Proteins; Soybean Proteins; Swine

2017
The metabolic analysis of psoriasis identifies the associated metabolites while providing computational models for the monitoring of the disease.
    Archives of dermatological research, 2017, Volume: 309, Issue:7

    Topics: Adult; Aged; Alkanes; Amino Acids; Carnitine; Computer Simulation; Dicarboxylic Acids; Female; Humans; Male; Metabolome; Middle Aged; Phosphatidylcholines; Phytol; Psoriasis; Urea; Young Adult

2017
DGAT1-Dependent Lipid Droplet Biogenesis Protects Mitochondrial Function during Starvation-Induced Autophagy.
    Developmental cell, 2017, 07-10, Volume: 42, Issue:1

    Topics: Amino Acids; Animals; Autophagy; Carnitine; Diacylglycerol O-Acyltransferase; Humans; Isotope Labeling; Lipid Droplets; Mechanistic Target of Rapamycin Complex 1; Mice; Mitochondria; Models, Biological; Multiprotein Complexes; Palmitic Acid; TOR Serine-Threonine Kinases; Triglycerides

2017
Selective and accurate C5 acylcarnitine quantitation by UHPLC-MS/MS: Distinguishing true isovaleric acidemia from pivalate derived interference.
    Journal of chromatography. B, Analytical technologies in the biomedical and life sciences, 2017, Sep-01, Volume: 1061-1062

    Topics: Amino Acid Metabolism, Inborn Errors; Carnitine; Chromatography, High Pressure Liquid; Humans; Isovaleryl-CoA Dehydrogenase; Limit of Detection; Reproducibility of Results; Tandem Mass Spectrometry

2017
Blood Metabolic Signatures of Body Mass Index: A Targeted Metabolomics Study in the EPIC Cohort.
    Journal of proteome research, 2017, 09-01, Volume: 16, Issue:9

    Topics: Adult; Amino Acids; Bile Duct Neoplasms; Biogenic Amines; Blood Proteins; Body Mass Index; Carcinoma; Carnitine; Case-Control Studies; Diabetes Mellitus; Europe; Gene Expression; Hexoses; Humans; Linear Models; Liver Neoplasms; Longitudinal Studies; Male; Metabolome; Middle Aged; Nutrition Assessment; Obesity; Phosphatidylcholines; Risk Factors; Sphingomyelins

2017
Acute and long-term administration of palmitoylcarnitine induces muscle-specific insulin resistance in mice.
    BioFactors (Oxford, England), 2017, Sep-10, Volume: 43, Issue:5

    Topics: Animals; Blood Glucose; Carbohydrate Metabolism; Carnitine; Dietary Fats; Energy Metabolism; Glucose; Humans; Insulin; Insulin Resistance; Lipid Metabolism; Mice; Muscle, Skeletal; Palmitoylcarnitine

2017
Decreased long-chain acylcarnitines from insufficient β-oxidation as potential early diagnostic markers for Parkinson's disease.
    Scientific reports, 2017, 08-04, Volume: 7, Issue:1

    Topics: Aged; Biomarkers; Carnitine; Cohort Studies; Fatty Acids; Female; Humans; Male; Metabolome; Metabolomics; Middle Aged; Mitochondria; Models, Biological; Muscle, Skeletal; Oxidation-Reduction; Parkinson Disease; ROC Curve; Severity of Illness Index

2017
Profiling of Acylcarnitines in First Episode Psychosis before and after Antipsychotic Treatment.
    Journal of proteome research, 2017, 10-06, Volume: 16, Issue:10

    Topics: Adolescent; Adult; Antipsychotic Agents; Biomarkers; Body Mass Index; Carnitine; Chromatography, Liquid; Female; Humans; Insulin Resistance; Interleukin-2; Interleukin-6; Male; Metabolomics; Middle Aged; Psychotic Disorders; Tandem Mass Spectrometry; Young Adult

2017
Acylcarnitines profile best predicts survival in horses with atypical myopathy.
    PloS one, 2017, Volume: 12, Issue:8

    Topics: Animals; Carnitine; Horse Diseases; Horses; Muscular Diseases; Prognosis; Survival Rate

2017
Comprehensive Analysis of Acylcarnitine Species in db/db Mouse Using a Novel Method of High-Resolution Parallel Reaction Monitoring Reveals Widespread Metabolic Dysfunction Induced by Diabetes.
    Analytical chemistry, 2017, 10-03, Volume: 89, Issue:19

    Topics: Animals; Carnitine; Chromatography, High Pressure Liquid; Diabetes Mellitus, Experimental; Limit of Detection; Male; Mice; Mice, Inbred C57BL; Principal Component Analysis; Tandem Mass Spectrometry

2017
The Liver as a Hub in Thermogenesis.
    Cell metabolism, 2017, 09-05, Volume: 26, Issue:3

    Topics: Adipose Tissue, Brown; Animals; Carnitine; Energy Metabolism; Lipids; Liver; Mice; Thermogenesis

2017
Global Analysis of Plasma Lipids Identifies Liver-Derived Acylcarnitines as a Fuel Source for Brown Fat Thermogenesis.
    Cell metabolism, 2017, Sep-05, Volume: 26, Issue:3

    Topics: Adipose Tissue, Brown; Aging; Animals; Body Temperature; Carnitine; Carnitine O-Palmitoyltransferase; Citric Acid Cycle; Cold Temperature; Fatty Acids; Gene Expression Regulation; Gene Knockdown Techniques; Hepatocyte Nuclear Factor 4; Lipids; Lipolysis; Liver; Mice; Phenotype; Thermogenesis; Time Factors

2017
Lower mitochondrial DNA and altered mitochondrial fuel metabolism in HIV-exposed uninfected infants in Cameroon.
    AIDS (London, England), 2017, 11-28, Volume: 31, Issue:18

    Topics: Amino Acids, Branched-Chain; Anti-Retroviral Agents; Cameroon; Carnitine; DNA, Mitochondrial; Energy Metabolism; HIV Infections; Humans; Infant; Mitochondria; Nevirapine; Real-Time Polymerase Chain Reaction; Tandem Mass Spectrometry; Zidovudine

2017
Fine Mapping and Functional Analysis Reveal a Role of SLC22A1 in Acylcarnitine Transport.
    American journal of human genetics, 2017, Oct-05, Volume: 101, Issue:4

    Topics: Alleles; Alternative Splicing; Animals; Biological Transport; Carnitine; Cells, Cultured; Cohort Studies; CRISPR-Cas Systems; Female; Gene Expression Regulation; Genome-Wide Association Study; High-Throughput Nucleotide Sequencing; Humans; Liver; Male; Metabolic Diseases; Metabolomics; Mice; Mice, Inbred BALB C; Mice, Inbred C57BL; Mice, Knockout; Organic Cation Transporter 1; Polymorphism, Single Nucleotide; Tissue Distribution

2017
Metabolomic profile of arterial stiffness in aged adults.
    Diabetes & vascular disease research, 2018, Volume: 15, Issue:1

    Topics: Age Factors; Aged; Aged, 80 and over; Aging; Amino Acids; Arterial Pressure; Biomarkers; Cardiovascular Diseases; Carnitine; Chromatography, High Pressure Liquid; Female; Gas Chromatography-Mass Spectrometry; Humans; Male; Manometry; Metabolomics; Middle Aged; Predictive Value of Tests; Prospective Studies; Pulse Wave Analysis; Risk Factors; Vascular Stiffness

2018
Effects of birth weight on profiles of dried blood amino-acids and acylcarnitines.
    Annals of clinical biochemistry, 2018, Volume: 55, Issue:1

    Topics: Amino Acids; Birth Weight; Carnitine; False Positive Reactions; Humans; Infant, Newborn; Neonatal Screening

2018
Maternal obesity and increased neonatal adiposity correspond with altered infant mesenchymal stem cell metabolism.
    JCI insight, 2017, 11-02, Volume: 2, Issue:21

    Topics: Adiposity; Amino Acids; Biomarkers; Carnitine; Colorado; Electron Transport; Energy Metabolism; Fatty Acids; Female; Gene Expression Regulation, Developmental; Humans; Infant; Infant, Newborn; Insulin; Lipid Metabolism; Lipids; Longitudinal Studies; Male; Maternal Nutritional Physiological Phenomena; Mesenchymal Stem Cells; Mitochondria; Mitochondrial Diseases; Muscle Cells; Obesity; Pregnancy

2017
Short communication: Circulating fatty acylcarnitines are elevated in overweight periparturient dairy cows in association with sphingolipid biomarkers of insulin resistance.
    Journal of dairy science, 2018, Volume: 101, Issue:1

    Topics: Animals; Biomarkers; Carnitine; Cattle; Cattle Diseases; Ceramides; Fatty Acids; Female; Insulin Resistance; Lactation; Lipid Metabolism; Overweight; Peripartum Period; Pregnancy; Sphingolipids

2018
[Rhabdomyolysis - may it be a metabolic myopathy? Case report and diagnostic algorithm].
    Orvosi hetilap, 2017, Volume: 158, Issue:47

    Topics: Algorithms; Carnitine; Diagnosis, Differential; Female; Humans; Middle Aged; Muscular Diseases; Rhabdomyolysis

2017
Similarities between acylcarnitine profiles in large for gestational age newborns and obesity.
    Scientific reports, 2017, 11-24, Volume: 7, Issue:1

    Topics: Cardiomyopathies; Carnitine; Female; Gestational Age; Humans; Hyperammonemia; Infant, Newborn; Infant, Small for Gestational Age; Male; Muscular Diseases; Obesity; Pregnancy

2017
Serum metabolites and risk of myocardial infarction and ischemic stroke: a targeted metabolomic approach in two German prospective cohorts.
    European journal of epidemiology, 2018, Volume: 33, Issue:1

    Topics: Aged; Amino Acids; Biomarkers; Brain Ischemia; Carnitine; Female; Germany; Humans; Incidence; Male; Metabolomics; Middle Aged; Myocardial Infarction; Phosphatidylcholines; Prospective Studies; Risk Factors; Serum; Stroke; Triglycerides

2018
Reference values of amino acids, acylcarnitines and succinylacetone by tandem mass spectrometry for use in newborn screening in southwest Colombia.
    Colombia medica (Cali, Colombia), 2017, Sep-30, Volume: 48, Issue:3

    Topics: Amino Acids; Biomarkers; Carnitine; Colombia; Cross-Sectional Studies; False Positive Reactions; Heptanoates; Humans; Infant, Newborn; Metabolism, Inborn Errors; Reference Values; Sensitivity and Specificity; Tandem Mass Spectrometry

2017
Plasma acylcarnitine concentrations reflect the acylcarnitine profile in cardiac tissues.
    Scientific reports, 2017, 12-13, Volume: 7, Issue:1

    Topics: Animals; Blood Glucose; Carnitine; Glucose Tolerance Test; Insulin; Liver; Male; Metabolic Diseases; Mice; Muscle, Skeletal; Myocardium; Random Allocation; Rats, Wistar; Running

2017
Propofol Related Infusion Syndrome: Ultrastructural Evidence for a Mitochondrial Disorder.
    Critical care medicine, 2018, Volume: 46, Issue:1

    Topics: Carnitine; Craniotomy; Hematoma, Subdural, Intracranial; Humans; Infusions, Intravenous; Male; Microscopy, Electron; Mitochondria, Heart; Mitochondria, Liver; Mitochondria, Muscle; Mitochondrial Diseases; Postoperative Complications; Propofol Infusion Syndrome; Young Adult

2018
Mice with an Oncogenic HRAS Mutation are Resistant to High-Fat Diet-Induced Obesity and Exhibit Impaired Hepatic Energy Homeostasis.
    EBioMedicine, 2018, Volume: 27

    Topics: Animals; Carnitine; Diet, High-Fat; Energy Metabolism; Face; Fatty Acids; Female; Gene Expression Regulation; Gene Knock-In Techniques; Glucose; Glutamine; Homeostasis; Hypertrophy; Kidney; Liver; Mice; Mitochondria; Mutation; Myocytes, Cardiac; Obesity; Oncogenes; Oxidation-Reduction; Phenotype; Proto-Oncogene Proteins p21(ras); ras Proteins; Weight Gain

2018
Non-invasive test using palmitate in patients with suspected fatty acid oxidation defects: disease-specific acylcarnitine patterns can help to establish the diagnosis.
    Orphanet journal of rare diseases, 2017, 12-21, Volume: 12, Issue:1

    Topics: Acyl-CoA Dehydrogenase; Adolescent; Analysis of Variance; Carnitine; Child; Child, Preschool; Fatty Acids; Female; Humans; Infant; Infant, Newborn; Lipid Metabolism, Inborn Errors; Male; Neonatal Screening; Palmitates; Reproducibility of Results; Tandem Mass Spectrometry

2017
Metabolomic analysis of 92 pulmonary embolism patients from a nested case-control study identifies metabolites associated with adverse clinical outcomes.
    Journal of thrombosis and haemostasis : JTH, 2018, Volume: 16, Issue:3

    Topics: Adolescent; Adult; Aged; Carnitine; Case-Control Studies; Fatty Acids; Female; Hemoglobins; Humans; Hypoxanthine; Inosine; Male; Metabolome; Middle Aged; Porphyrins; Prospective Studies; Pulmonary Embolism; Purines; Risk Assessment; Treatment Outcome; Tricarboxylic Acids; Young Adult

2018
Association of Acylcarnitines With Left Ventricular Remodeling in Patients With Severe Aortic Stenosis Undergoing Transcatheter Aortic Valve Replacement.
    JAMA cardiology, 2018, 03-01, Volume: 3, Issue:3

    Topics: Aged, 80 and over; Aortic Valve Stenosis; Biomarkers; Carnitine; Choline; Diabetes Complications; Female; Humans; Hypertension; Kynurenine; Male; Renal Insufficiency, Chronic; Stroke Volume; Transcatheter Aortic Valve Replacement; Ventricular Remodeling

2018
A quantitative multimodal metabolomic assay for colorectal cancer.
    BMC cancer, 2018, 01-04, Volume: 18, Issue:1

    Topics: Adenoma; Aged; Amino Acids; Biomarkers, Tumor; Carnitine; Colorectal Neoplasms; Early Detection of Cancer; Female; Humans; Male; Mass Spectrometry; Metabolome; Metabolomics; Middle Aged; Phosphatidylcholines; Sphingomyelins

2018
Biochemical signatures mimicking multiple carboxylase deficiency in children with mutations in MT-ATP6.
    Mitochondrion, 2019, Volume: 44

    Topics: Adolescent; Carnitine; Child; Child, Preschool; Cohort Studies; Female; Genetic Testing; Humans; Infant; Infant, Newborn; Male; Mitochondrial Proton-Translocating ATPases; Multiple Carboxylase Deficiency; Mutation; Retrospective Studies; Young Adult

2019
Medium- and long-chain acylcarnitines are associated with osteoarthritis severity and arterial stiffness in end-stage osteoarthritis patients: a case-control study.
    International journal of rheumatic diseases, 2018, Volume: 21, Issue:6

    Topics: Aged; Biomarkers; Carnitine; Case-Control Studies; Down-Regulation; Female; Humans; Male; Metabolomics; Middle Aged; Osteoarthritis, Hip; Osteoarthritis, Knee; Principal Component Analysis; Prospective Studies; Pulse Wave Analysis; Severity of Illness Index; Vascular Stiffness

2018
Medium-chain acyl-CoA dehydrogenase deficiency: Two novel ACADM mutations identified in a retrospective screening.
    The Journal of international medical research, 2018, Volume: 46, Issue:4

    Topics: Acyl-CoA Dehydrogenase; Carboxylic Acids; Carnitine; Dried Blood Spot Testing; Female; Humans; Infant; Infant, Newborn; Lipid Metabolism, Inborn Errors; Male; Mutation; Neonatal Screening; Retrospective Studies

2018
Lysosomal acid lipase regulates fatty acid channeling in brown adipose tissue to maintain thermogenesis.
    Biochimica et biophysica acta. Molecular and cell biology of lipids, 2018, Volume: 1863, Issue:4

    Topics: Acetyl Coenzyme A; Adipocytes, Brown; Adipose Tissue, Brown; Animals; Autophagy; Body Temperature; Carnitine; Cold Temperature; Disease Progression; Dyslipidemias; Energy Metabolism; Fatty Acids; Glucose; Hypothermia, Induced; Lipid Droplets; Lipolysis; Male; Mice, Inbred C57BL; Muscles; Oxidation-Reduction; Oxygen Consumption; Sterol Esterase; Thermogenesis; Uncoupling Protein 1

2018
Septic Shock Nonsurvivors Have Persistently Elevated Acylcarnitines Following Carnitine Supplementation.
    Shock (Augusta, Ga.), 2018, Volume: 49, Issue:4

    Topics: Aged; Carnitine; Fatty Acids; Female; Humans; Insulin; Magnetic Resonance Spectroscopy; Male; Middle Aged; Shock, Septic

2018
Lipidomic analysis of serum samples from migraine patients.
    Lipids in health and disease, 2018, Feb-02, Volume: 17, Issue:1

    Topics: Adult; Biomarkers; Carnitine; Ceramides; Chromatography, Liquid; Female; Humans; Lipids; Lysophosphatidylcholines; Lysophospholipids; Male; Mass Spectrometry; Migraine Disorders

2018
Metabolomics signatures associated with an oral glucose challenge in pregnant women.
    Diabetes & metabolism, 2019, Volume: 45, Issue:1

    Topics: Adolescent; Adult; Bile Acids and Salts; Blood Glucose; Carnitine; Diabetes, Gestational; Diglycerides; Fatty Acids; Female; Glucose; Glucose Tolerance Test; Humans; Lipid Metabolism; Metabolomics; Pregnancy; Young Adult

2019
Cord Blood Metabolic Signatures of Birth Weight: A Population-Based Study.
    Journal of proteome research, 2018, 03-02, Volume: 17, Issue:3

    Topics: Birth Weight; Carnitine; Chromatography, High Pressure Liquid; Cohort Studies; Docosahexaenoic Acids; Environmental Exposure; Europe; Female; Fetal Blood; Fetal Development; Fetus; Humans; Indoles; Infant, Newborn; Male; Mass Spectrometry; Metabolome; Particulate Matter; Phosphatidylcholines; Pregnancy; Progesterone; Prostaglandins; Reference Standards; Tryptophan; Vitamin A

2018
Kruppel-like factor 15 is required for the cardiac adaptive response to fasting.
    PloS one, 2018, Volume: 13, Issue:2

    Topics: Adaptation, Physiological; Animals; Carnitine; DNA-Binding Proteins; Echocardiography; Fasting; Heart; Kruppel-Like Transcription Factors; Lipid Metabolism; Male; Mice; Mice, Knockout; Mitochondria, Heart; Transcription Factors

2018
Interventional Left Atrial Appendage Closure Affects the Metabolism of Acylcarnitines.
    International journal of molecular sciences, 2018, Feb-07, Volume: 19, Issue:2

    Topics: Aged; Aged, 80 and over; Atrial Appendage; Atrial Fibrillation; Biomarkers; Carnitine; Cohort Studies; Endovascular Procedures; Female; Humans; Male; Metabolomics; Prospective Studies; Regression Analysis; Stroke; Vascular Closure Devices

2018
Insulin-resistance in glycogen storage disease type Ia: linking carbohydrates and mitochondria?
    Journal of inherited metabolic disease, 2018, Volume: 41, Issue:6

    Topics: Acids; Adolescent; Adult; Antiporters; Biomarkers; Carnitine; Case-Control Studies; Child; Child, Preschool; Female; Glucose-6-Phosphatase; Glycogen Storage Disease Type I; Humans; Insulin; Insulin Resistance; Linear Models; Male; Mitochondrial Diseases; Monosaccharide Transport Proteins; Multivariate Analysis; Urinalysis; Young Adult

2018
Elevated Medium-Chain Acylcarnitines Are Associated With Gestational Diabetes Mellitus and Early Progression to Type 2 Diabetes and Induce Pancreatic β-Cell Dysfunction.
    Diabetes, 2018, Volume: 67, Issue:5

    Topics: Adult; Animals; Carnitine; Case-Control Studies; Cell Respiration; Diabetes Mellitus, Type 2; Diabetes, Gestational; Disease Progression; Female; Glucose Intolerance; Humans; Insulin; Insulin Secretion; Insulin-Secreting Cells; Islets of Langerhans; Mice; Postpartum Period; Pregnancy

2018
CPT2 downregulation adapts HCC to lipid-rich environment and promotes carcinogenesis via acylcarnitine accumulation in obesity.
    Gut, 2018, Volume: 67, Issue:8

    Topics: Adult; Aged; Animals; Carcinoma, Hepatocellular; Carnitine; Carnitine O-Palmitoyltransferase; Case-Control Studies; Disease Models, Animal; Female; Humans; Liver Neoplasms; Male; Mice; Middle Aged; Non-alcoholic Fatty Liver Disease; Obesity

2018
A Plasma Metabolomic Signature of the Exfoliation Syndrome Involves Amino Acids, Acylcarnitines, and Polyamines.
    Investigative ophthalmology & visual science, 2018, 02-01, Volume: 59, Issue:2

    Topics: Aged; Amino Acids; Biomarkers; Carnitine; Exfoliation Syndrome; Female; Glaucoma, Open-Angle; Humans; Male; Mass Spectrometry; Metabolome; Metabolomics; Polyamines

2018
Obesity-Related Metabolomic Profiles and Discrimination of Metabolically Unhealthy Obesity.
    Journal of proteome research, 2018, 04-06, Volume: 17, Issue:4

    Topics: Adolescent; Adult; Carnitine; Case-Control Studies; Humans; Iran; Lysophosphatidylcholines; Metabolomics; Middle Aged; Obesity; Phenotype; Risk Assessment; Young Adult

2018
Arsenic induces cardiac rhythm dysfunction and acylcarnitines metabolism perturbation in rats.
    Toxicology mechanisms and methods, 2018, Volume: 28, Issue:6

    Topics: Animals; Arsenites; Carnitine; Dose-Response Relationship, Drug; Electrocardiography; Environmental Pollutants; Heart Rate; Long QT Syndrome; Male; Rats, Sprague-Dawley; Sodium Compounds

2018
Differential requirements for processing and transport of short-chain versus long-chain O-acylcarnitines in Pseudomonas aeruginosa.
    Microbiology (Reading, England), 2018, Volume: 164, Issue:4

    Topics: ATP-Binding Cassette Transporters; Biological Transport, Active; Carnitine; Carrier Proteins; Extracellular Space; Hydrolases; Hydrolysis; Models, Biological; Mutation; Pseudomonas aeruginosa

2018
Use of Metabolomics as a Complementary Omic Approach to Implement Risk Criteria for First-Degree Relatives of Gastric Cancer Patients.
    International journal of molecular sciences, 2018, Mar-07, Volume: 19, Issue:3

    Topics: Age Factors; Biomarkers, Tumor; Carnitine; Computational Biology; Early Detection of Cancer; Family; Female; Helicobacter Infections; Humans; Male; Metabolome; Metabolomics; Risk Assessment; ROC Curve; Sphingomyelins; Stomach Neoplasms

2018
Adaptive remodeling of skeletal muscle energy metabolism in high-altitude hypoxia: Lessons from AltitudeOmics.
    The Journal of biological chemistry, 2018, 05-04, Volume: 293, Issue:18

    Topics: Acclimatization; Altitude; Altitude Sickness; Amino Acids; Carnitine; Energy Metabolism; Fatty Acids; Female; Glycolysis; Healthy Volunteers; Humans; Male; Metabolome; Mitochondria, Muscle; Muscle Proteins; Muscle, Skeletal; Oxidation-Reduction; Pentose Phosphate Pathway; Phosphorylation; Proteolysis; Proteomics; Purine Nucleotides; Random Allocation; Stress, Physiological; Young Adult

2018
Targeted metabolomic profiling in rat tissues reveals sex differences.
    Scientific reports, 2018, 03-16, Volume: 8, Issue:1

    Topics: Amino Acids; Animals; Carnitine; Cluster Analysis; Discriminant Analysis; Female; Kidney; Liver; Male; Metabolomics; Multivariate Analysis; Myocardium; Organ Specificity; Principal Component Analysis; Rats; Rats, Sprague-Dawley; Sex Characteristics

2018
Phosphorylation of MCAD selectively rescues PINK1 deficiencies in behavior and metabolism.
    Molecular biology of the cell, 2018, 05-15, Volume: 29, Issue:10

    Topics: Acyl-CoA Dehydrogenase; Adenosine Triphosphate; Amino Acid Sequence; Amino Acids; Animals; Animals, Genetically Modified; Carnitine; Drosophila melanogaster; Drosophila Proteins; Oxidation-Reduction; Phenotype; Phosphorylation; Phosphoserine; Protein Serine-Threonine Kinases

2018
Orange juice affects acylcarnitine metabolism in healthy volunteers as revealed by a mass-spectrometry based metabolomics approach.
    Food research international (Ottawa, Ont.), 2018, Volume: 107

    Topics: Adult; Carnitine; Chromatography, Liquid; Citrus sinensis; Female; Fruit and Vegetable Juices; Gas Chromatography-Mass Spectrometry; Humans; Male; Mass Spectrometry; Metabolomics; Middle Aged; Reference Values; Young Adult

2018
Risk of gestational diabetes mellitus in relation to plasma concentrations of amino acids and acylcarnitines: A nested case-control study.
    Diabetes research and clinical practice, 2018, Volume: 140

    Topics: Adult; Amino Acids; Biomarkers; Carnitine; Case-Control Studies; Diabetes, Gestational; Female; Humans; Pregnancy

2018
Carnitine Deficiency in Chinese Children with Epilepsy on Valproate Monotherapy.
    Indian pediatrics, 2018, 03-15, Volume: 55, Issue:3

    Topics: Anticonvulsants; Beijing; Carnitine; Case-Control Studies; Child, Preschool; Deficiency Diseases; Epilepsy; Female; Humans; Infant; Male; Valproic Acid

2018
Strategy for Comprehensive Identification of Acylcarnitines Based on Liquid Chromatography-High-Resolution Mass Spectrometry.
    Analytical chemistry, 2018, 05-01, Volume: 90, Issue:9

    Topics: Adult; Animals; Carnitine; Chromatography, Liquid; Humans; Molecular Structure; Rats; Tandem Mass Spectrometry

2018
PPARα Mediates the Hepatoprotective Effects of Nutmeg.
    Journal of proteome research, 2018, 05-04, Volume: 17, Issue:5

    Topics: Animals; Carnitine; Chemical and Drug Induced Liver Injury; Lipids; Metabolomics; Mice; Mice, Knockout; Myristica; Oxidative Stress; PPAR alpha; Protective Agents; Thioacetamide

2018
An unusually high frequency of SCAD deficiency caused by two pathogenic variants in the ACADS gene and its relationship to the ethnic structure in Slovakia.
    BMC medical genetics, 2018, 04-20, Volume: 19, Issue:1

    Topics: Acyl-CoA Dehydrogenase; Butyryl-CoA Dehydrogenase; Carnitine; Ethnicity; Female; Gene Frequency; Genetic Testing; Humans; Infant, Newborn; Lipid Metabolism, Inborn Errors; Male; Mutation; Neonatal Screening; Polymorphism, Restriction Fragment Length; Sequence Analysis, DNA; Slovakia

2018
Theacrine protects against nonalcoholic fatty liver disease by regulating acylcarnitine metabolism.
    Metabolism: clinical and experimental, 2018, Volume: 85

    Topics: Animals; Apolipoproteins E; Carnitine; Diet, High-Fat; Energy Metabolism; Male; Mice; Mice, Knockout; Non-alcoholic Fatty Liver Disease; Oleic Acid; Plant Extracts; Protective Agents; Signal Transduction; Uric Acid

2018
Mutations in PPCS, Encoding Phosphopantothenoylcysteine Synthetase, Cause Autosomal-Recessive Dilated Cardiomyopathy.
    American journal of human genetics, 2018, 06-07, Volume: 102, Issue:6

    Topics: Amino Acid Sequence; Animals; Biosynthetic Pathways; Cardiomyopathy, Dilated; Carnitine; Child, Preschool; Coenzyme A; Demography; Drosophila; Enzyme Stability; Female; Fibroblasts; Genes, Recessive; Heart; High-Throughput Nucleotide Sequencing; Homozygote; Humans; Infant; Infant, Newborn; Magnetic Resonance Imaging; Male; Mutation; Pantetheine; Pedigree; Peptide Synthases; Reproducibility of Results; Saccharomyces cerevisiae

2018
Atypical myopathy in Père David's deer (Elaphurus davidianus) associated with ingestion of hypoglycin A.
    Journal of animal science, 2018, Jul-28, Volume: 96, Issue:8

    Topics: Acer; Animal Husbandry; Animals; Austria; Carnitine; Deer; Eating; Female; Germany; Hypoglycins; Male; Muscular Diseases; Seasons

2018
Evaluation ofserum free carnitine/acylcarnitine levels and left ventricular systolic functions in children with idiopathic epilepsy receiving valproic acid.
    Clinical neurology and neurosurgery, 2018, Volume: 170

    Topics: Adolescent; Anticonvulsants; Biomarkers; Carnitine; Child; Child, Preschool; Electrocardiography; Epilepsy; Female; Humans; Male; Valproic Acid; Ventricular Function, Left

2018
Remodeling of skeletal muscle mitochondrial proteome with high-fat diet involves greater changes to β-oxidation than electron transfer proteins in mice.
    American journal of physiology. Endocrinology and metabolism, 2018, 10-01, Volume: 315, Issue:4

    Topics: Adenosine Triphosphate; Animals; Carnitine; Cell Respiration; Chromatography, Liquid; Diet, Fat-Restricted; Diet, High-Fat; Electron Transport; Electron-Transferring Flavoproteins; Insulin Resistance; Lipid Metabolism; Male; Mass Spectrometry; Mice; Mice, Inbred C57BL; Mitochondria, Muscle; Mitochondrial Proteins; Muscle, Skeletal; Oxidation-Reduction; Oxygen Consumption; Protein Processing, Post-Translational; Proteome; Proteomics; Tandem Mass Spectrometry

2018
An Exploratory Analysis of Potential New Biomarkers of Cognitive Function.
    The journals of gerontology. Series A, Biological sciences and medical sciences, 2019, 02-15, Volume: 74, Issue:3

    Topics: Aged; Amino Acids; Biomarkers; Carnitine; Cognitive Dysfunction; Cross-Sectional Studies; Female; Geriatric Assessment; Humans; Hydroxybutyrates; Longitudinal Studies; Male; Middle Aged; Neuropsychological Tests

2019
Acylcarnitine Profiles in Plasma and Tissues of Hyperglycemic NZO Mice Correlate with Metabolite Changes of Human Diabetes.
    Journal of diabetes research, 2018, Volume: 2018

    Topics: Adipose Tissue; Animals; Blood Glucose; Carnitine; Fatty Acids, Nonesterified; Female; Hyperglycemia; Insulin; Kidney; Liver; Male; Mice; Mice, Obese; Muscle, Skeletal; Myocardium; Obesity; Triglycerides

2018
Glucose challenge metabolomics implicates medium-chain acylcarnitines in insulin resistance.
    Scientific reports, 2018, 06-06, Volume: 8, Issue:1

    Topics: 3T3-L1 Cells; Aged; Animals; Carnitine; Diabetes Mellitus, Type 2; Glucose; Glucose Tolerance Test; Humans; Insulin Resistance; Lipolysis; Male; Metabolomics; Mice

2018
A pilot data analysis of a metabolomic HPLC-MS/MS study of patients with COPD.
    Advances in clinical and experimental medicine : official organ Wroclaw Medical University, 2018, Volume: 27, Issue:4

    Topics: Amino Acids; Carnitine; Case-Control Studies; Chromatography, High Pressure Liquid; Data Analysis; Humans; Pulmonary Disease, Chronic Obstructive; Tandem Mass Spectrometry

2018
Acyl-carnitine, C5DC, and C26 as potential biomarkers for diagnosis of autism spectrum disorder in children.
    Psychiatry research, 2018, Volume: 267

    Topics: Autism Spectrum Disorder; Biomarkers; Carnitine; Child; Child, Preschool; China; Female; Humans; Intelligence; Male; Neurodevelopmental Disorders

2018
Persistent hypoglycemia associated with lipid storage myopathy in a paint foal.
    Journal of veterinary internal medicine, 2018, Volume: 32, Issue:4

    Topics: Animals; Carnitine; Diagnosis, Differential; Fatal Outcome; Female; Horse Diseases; Horses; Hypoglycemia; Lipid Metabolism, Inborn Errors; Multiple Acyl Coenzyme A Dehydrogenase Deficiency; Muscular Dystrophies

2018
Carnitine Precursors and Short-Chain Acylcarnitines in Water Buffalo Milk.
    Journal of agricultural and food chemistry, 2018, Aug-01, Volume: 66, Issue:30

    Topics: Animals; Buffaloes; Carnitine; Cattle; Female; Milk; Tandem Mass Spectrometry

2018
Clinical Features of Multiple Acyl-CoA Dehydrogenase Deficiency With ETFDH Variants in the First Korean Cases.
    Annals of laboratory medicine, 2018, Volume: 38, Issue:6

    Topics: Asian People; Base Sequence; Carnitine; Dietary Supplements; DNA Mutational Analysis; Electron-Transferring Flavoproteins; Gene Deletion; Humans; Infant, Newborn; Iron-Sulfur Proteins; Male; Multiple Acyl Coenzyme A Dehydrogenase Deficiency; Oxidoreductases Acting on CH-NH Group Donors; Pedigree; Republic of Korea; Riboflavin; Tandem Mass Spectrometry

2018
Tissue acylcarnitine status in a mouse model of mitochondrial β-oxidation deficiency during metabolic decompensation due to influenza virus infection.
    Molecular genetics and metabolism, 2018, Volume: 125, Issue:1-2

    Topics: Acyl-CoA Dehydrogenase, Long-Chain; Animals; Cardiomyopathies; Carnitine; Congenital Bone Marrow Failure Syndromes; Disease Models, Animal; Fatty Acids; Female; Humans; Hypoglycemia; Lipid Metabolism, Inborn Errors; Lipid Peroxidation; Liver; Liver Failure; Metabolic Diseases; Mice; Mitochondrial Diseases; Muscle, Skeletal; Muscular Diseases; Myocardium; Oxidation-Reduction

2018
The effect of high-fat diet and inhibition of ceramide production on insulin action in liver.
    Journal of cellular physiology, 2019, Volume: 234, Issue:2

    Topics: Animals; Biomarkers; Blood Glucose; Carnitine; Carnitine O-Palmitoyltransferase; Ceramides; Diet, High-Fat; Enzyme Inhibitors; Fatty Acids, Monounsaturated; Humans; Insulin; Insulin Resistance; Liver; Nerve Tissue Proteins; Rats, Wistar; Serine C-Palmitoyltransferase; Signal Transduction

2019
Metabolite profiling of plasma in patients with ossification of the posterior longitudinal ligament.
    Journal of orthopaedic science : official journal of the Japanese Orthopaedic Association, 2018, Volume: 23, Issue:6

    Topics: Aged; Carnitine; Case-Control Studies; Cervical Vertebrae; Fatty Acids; Humans; Male; Metabolome; Middle Aged; Ossification of Posterior Longitudinal Ligament; Palmitoylcarnitine; Thiazolidines; Thyroxine

2018
Alterations in plasma acylcarnitine and amino acid profiles may indicate poor nutrition during the suckling period due to maternal intake of an unbalanced diet and may predict later metabolic dysfunction.
    FASEB journal : official publication of the Federation of American Societies for Experimental Biology, 2019, Volume: 33, Issue:1

    Topics: Amino Acids; Animals; Animals, Suckling; Carnitine; Diet; Female; Lactation; Lipid Metabolism; Liver; Male; Maternal Nutritional Physiological Phenomena; Metabolic Diseases; Models, Animal; Multivariate Analysis; Nutritional Status; Pregnancy; Prenatal Exposure Delayed Effects; Rats

2019
Dietary stearic acid regulates mitochondria in vivo in humans.
    Nature communications, 2018, 08-07, Volume: 9, Issue:1

    Topics: Adult; Beverages; Carnitine; Case-Control Studies; Cross-Over Studies; Diabetes Mellitus; Diet; Fatty Acids; Female; Humans; Male; Middle Aged; Mitochondria; Mitochondrial Dynamics; Musa; Oxygen; Stearic Acids

2018
Selective Screening of Fatty Acids Oxidation Defects and Organic Acidemias by Liquid Chromatography/tandem Mass Spectrometry Acylcarnitine Analysis in Brazilian Patients.
    Archives of medical research, 2018, Volume: 49, Issue:3

    Topics: Acyl-CoA Dehydrogenase; Amino Acid Metabolism, Inborn Errors; Brain Diseases, Metabolic; Brazil; Carnitine; Child, Preschool; Chromatography, Liquid; Fatty Acids; Female; Glutarates; Glutaryl-CoA Dehydrogenase; Humans; Infant, Newborn; Lipid Metabolism, Inborn Errors; Male; Mass Screening; Oxidation-Reduction; Prevalence; Tandem Mass Spectrometry; Young Adult

2018
Clinical, Biochemical, and Genetic Findings of Cystinuria in Chinese Children.
    Clinical laboratory, 2018, Jul-01, Volume: 64, Issue:7

    Topics: Adolescent; Amino Acid Transport Systems, Basic; Amino Acid Transport Systems, Neutral; Amino Acids; Asian People; Carnitine; Child; Child, Preschool; China; Cystinuria; Family Health; Female; Humans; Infant; Infant, Newborn; Male; Mutation

2018
Biochemical profile in an infant with neonatal hemochromatosis shows evidence of impairment of mitochondrial long-chain fatty acid oxidation.
    Clinical and molecular hepatology, 2019, Volume: 25, Issue:1

    Topics: Amino Acids; Carnitine; Cluster Analysis; Fatty Acids; Hemochromatosis; Humans; Infant; Lipid Peroxidation; Liver; Male; Mass Spectrometry; Mitochondria

2019
Alterations in levels of intermediate-chained acylcarnitines associate with weight-gain following reestablishment of euthyroidism in Graves' disease.
    Endocrine, 2019, Volume: 63, Issue:1

    Topics: Adult; Antithyroid Agents; Carnitine; Female; Graves Disease; Humans; Iodine Radioisotopes; Male; Middle Aged; Predictive Value of Tests; Weight Gain

2019
Nonhuman Primates with Acute Radiation Syndrome: Results from a Global Serum Metabolomics Study after 7.2 Gy Total-Body Irradiation.
    Radiation research, 2018, Volume: 190, Issue:6

    Topics: Acute Radiation Syndrome; Amino Acids; Animals; Carnitine; Cytokines; Humans; Lipids; Macaca mulatta; Mass Spectrometry; Metabolome; Metabolomics; Primates; Purines; Radiation, Ionizing; Whole-Body Irradiation

2018
Dysregulated serum metabolites in staging of hepatocellular carcinoma.
    Clinical biochemistry, 2018, Volume: 61

    Topics: Adult; Aged; Biomarkers, Tumor; Carcinoma, Hepatocellular; Carnitine; China; Cohort Studies; Docosahexaenoic Acids; Female; Humans; Linolenic Acids; Liver Neoplasms; Lymphatic Metastasis; Lysophosphatidylcholines; Male; Middle Aged; Neoplasm Grading; Neoplasm Staging; Principal Component Analysis; Prognosis; Proof of Concept Study; Sensitivity and Specificity; Up-Regulation; Young Adult

2018
The saturation degree of fatty acids and their derived acylcarnitines determines the direct effect of metabolically active thyroid hormones on insulin sensitivity in skeletal muscle cells.
    FASEB journal : official publication of the Federation of American Societies for Experimental Biology, 2019, Volume: 33, Issue:2

    Topics: Animals; Biological Transport; Carnitine; Cell Line; Fatty Acids; Glycolysis; Insulin; Insulin Resistance; Mitochondria, Muscle; Muscle, Skeletal; Oxidation-Reduction; Rats; Signal Transduction; Thyroid Hormones

2019
Cord Blood Metabolites Associated with Newborn Adiposity and Hyperinsulinemia.
    The Journal of pediatrics, 2018, Volume: 203

    Topics: Adiposity; Adult; Amino Acids; C-Peptide; Carnitine; Cross-Sectional Studies; Female; Fetal Blood; Humans; Hyperinsulinism; Infant, Newborn; Leptin; Male; Multivariate Analysis; Principal Component Analysis

2018
Urinary and plasma metabolite differences detected by HPLC-ESI-QTOF-MS in systemic sclerosis patients.
    Journal of pharmaceutical and biomedical analysis, 2019, Jan-05, Volume: 162

    Topics: Acylation; Adult; Aged; Arachidonic Acids; Biomarkers; Carnitine; Case-Control Studies; Chromatography, High Pressure Liquid; Endocannabinoids; Female; Glycerides; Glycine; Humans; Male; Metabolomics; Middle Aged; Predictive Value of Tests; Prognosis; Reproducibility of Results; Scleroderma, Systemic; Spectrometry, Mass, Electrospray Ionization; Urinalysis

2019
Performance of LC/MS/MS in Analyzing Multiple Amino Acids and Acylcarnitines in Dried Blood Spot of Very Low Birth Weight, Low Birth Weight, and Normal Weight Neonates.
    Clinical laboratory, 2018, Sep-01, Volume: 64, Issue:9

    Topics: Amino Acids; Biomarkers; Birth Weight; Carnitine; Case-Control Studies; Chromatography, High Pressure Liquid; Dried Blood Spot Testing; Female; Humans; Infant Nutritional Physiological Phenomena; Infant, Newborn; Infant, Very Low Birth Weight; Male; Neonatal Screening; Nutritional Status; Predictive Value of Tests; Reproducibility of Results; Tandem Mass Spectrometry

2018
A Non-Targeted LC-MS Profiling Reveals Elevated Levels of Carnitine Precursors and Trimethylated Compounds in the Cord Plasma of Pre-Eclamptic Infants.
    Scientific reports, 2018, 10-02, Volume: 8, Issue:1

    Topics: Adult; Biomarkers; Carnitine; Case-Control Studies; Chromatography, Liquid; Creatine; Cross-Sectional Studies; Female; Fetal Blood; Humans; Infant, Newborn; Metabolomics; Phosphatidylcholines; Pre-Eclampsia; Pregnancy; Principal Component Analysis; Putrescine; Pyridoxic Acid; Tandem Mass Spectrometry; Tryptophan; Urea

2018
Metabolomic profiles and development of metabolic risk during the pubertal transition: a prospective study in the ELEMENT Project.
    Pediatric research, 2019, Volume: 85, Issue:3

    Topics: Acetylcarnitine; Adolescent; Amino Acids, Branched-Chain; Aminoisobutyric Acids; Asparagine; Biomarkers; Blood Glucose; Body Composition; Body Mass Index; C-Peptide; Carnitine; Child; Cholesterol; Female; Humans; Hydroxyproline; Hyperglycemia; Insulin Resistance; Leptin; Male; Metabolome; Ornithine; Prospective Studies; Puberty; Regression Analysis; Risk Factors

2019
Mitochondrial complex IV deficiency caused by a novel frameshift variant in MT-CO2 associated with myopathy and perturbed acylcarnitine profile.
    European journal of human genetics : EJHG, 2019, Volume: 27, Issue:2

    Topics: Adolescent; Carnitine; Codon, Terminator; Electron Transport Complex IV; Female; Frameshift Mutation; Humans; Mitochondria, Muscle; Mitochondrial Diseases; Muscular Diseases

2019
The Carnitine Shuttle Pathway is Altered in Patients With Neovascular Age-Related Macular Degeneration.
    Investigative ophthalmology & visual science, 2018, 10-01, Volume: 59, Issue:12

    Topics: Aged; Carnitine; Choroidal Neovascularization; Chromatography, Liquid; Fatty Acids; Female; Humans; Male; Metabolic Networks and Pathways; Metabolomics; Tandem Mass Spectrometry; Wet Macular Degeneration

2018
Acylcarnitine profiles in serum and muscle of dairy cows receiving conjugated linoleic acids or a control fat supplement during early lactation.
    Journal of dairy science, 2019, Volume: 102, Issue:1

    Topics: Animals; Carnitine; Cattle; Dietary Supplements; Fatty Acids; Female; Lactation; Linoleic Acids, Conjugated; Milk; Muscle, Skeletal; Parturition; Pregnancy

2019
3D MALDI mass spectrometry imaging reveals specific localization of long-chain acylcarnitines within a 10-day time window of spinal cord injury.
    Scientific reports, 2018, 10-31, Volume: 8, Issue:1

    Topics: Animals; Carnitine; Image Processing, Computer-Assisted; Imaging, Three-Dimensional; Macrophages; Male; Microglia; Rats; Rats, Wistar; Spectrometry, Mass, Matrix-Assisted Laser Desorption-Ionization; Spinal Cord Injuries

2018
Plasma Acylcarnitines and Risk of Type 2 Diabetes in a Mediterranean Population at High Cardiovascular Risk.
    The Journal of clinical endocrinology and metabolism, 2019, 05-01, Volume: 104, Issue:5

    Topics: Aged; Aged, 80 and over; Biomarkers; Cardiovascular Diseases; Carnitine; Case-Control Studies; Diabetes Mellitus, Type 2; Diet, Mediterranean; Female; Follow-Up Studies; Humans; Incidence; Male; Metabolomics; Middle Aged; Prognosis; Prospective Studies; Risk Factors; ROC Curve

2019
Metabolomic Signature in Sera of Multiple Sclerosis Patients during Pregnancy.
    International journal of molecular sciences, 2018, Nov-14, Volume: 19, Issue:11

    Topics: Adult; Amino Acids; Biomarkers; Carnitine; Case-Control Studies; Female; Gonadal Steroid Hormones; Humans; Hydrocortisone; Metabolome; Multiple Sclerosis; Pregnancy; Pregnancy Complications; Sphingolipids

2018
Plasma metabolites in first episode psychoses.
    Schizophrenia research, 2019, Volume: 206

    Topics: Adolescent; Adult; Biomarkers; Bipolar Disorder; Carnitine; Female; Humans; Lysophosphatidylcholines; Male; Phosphatidylcholines; Psychiatric Status Rating Scales; Psychotic Disorders; Schizophrenia; Young Adult

2019
Yield of laboratory testing in pediatric ketogenic diet patients: Critical assessment of abnormal results and impact on clinical care.
    Epilepsy research, 2019, Volume: 149

    Topics: Adolescent; Carnitine; Child; Child, Preschool; Clinical Laboratory Techniques; Cohort Studies; Critical Care Outcomes; Diet, Ketogenic; Epilepsy; Female; Humans; Infant; Male; Phosphorus

2019
Effects of caloric restriction on neuropathic pain, peripheral nerve degeneration and inflammation in normometabolic and autophagy defective prediabetic Ambra1 mice.
    PloS one, 2018, Volume: 13, Issue:12

    Topics: Adaptor Proteins, Signal Transducing; Amino Acids; AMP-Activated Protein Kinases; Animals; Autophagy; Caloric Restriction; Carnitine; Cytokines; Energy Metabolism; Glucose; Heterozygote; Inflammation; Insulin Resistance; Male; Mice; Mice, Transgenic; Nerve Degeneration; Neuralgia; Prediabetic State; Schwann Cells

2018
Peroxisomes can oxidize medium- and long-chain fatty acids through a pathway involving ABCD3 and HSD17B4.
    FASEB journal : official publication of the Federation of American Societies for Experimental Biology, 2019, Volume: 33, Issue:3

    Topics: Animals; ATP-Binding Cassette Transporters; Carnitine; Carnitine O-Palmitoyltransferase; CRISPR-Cas Systems; Fatty Acids; HEK293 Cells; Humans; Lauric Acids; Membrane Proteins; Mice; Mice, Knockout; Mitochondria; Oxidation-Reduction; Palmitic Acid; Peroxisomal Bifunctional Enzyme; Peroxisomal Multifunctional Protein-2; Peroxisomes; Recombinant Proteins

2019
Erythrocyte PUFAs, circulating acylcarnitines, and metabolic syndrome risk: a prospective study in Chinese.
    Journal of lipid research, 2019, Volume: 60, Issue:2

    Topics: Aged; Carnitine; China; Erythrocytes; Fatty Acids, Unsaturated; Female; Humans; Male; Metabolic Syndrome; Middle Aged; Prospective Studies; Risk Factors

2019
Analysis of amino acid and acyl carnitine profiles in maternal and fetal serum from preeclampsia patients.
    The journal of maternal-fetal & neonatal medicine : the official journal of the European Association of Perinatal Medicine, the Federation of Asia and Oceania Perinatal Societies, the International Society of Perinatal Obstetricians, 2020, Volume: 33, Issue:16

    Topics: Adult; Amino Acids; Biomarkers; Carnitine; Case-Control Studies; Chromatography, High Pressure Liquid; Female; Fetal Blood; Humans; Infant, Newborn; Pre-Eclampsia; Pregnancy; Retrospective Studies

2020
Zonisamide Administration Improves Fatty Acid β-Oxidation in Parkinson's Disease.
    Cells, 2018, 12-29, Volume: 8, Issue:1

    Topics: Aged; Aged, 80 and over; Antiparkinson Agents; Carnitine; Fatty Acids; Female; Humans; Levodopa; Male; Mental Status and Dementia Tests; Metabolome; Middle Aged; Mitochondria; Oxidation-Reduction; Parkinson Disease; Pilot Projects; Severity of Illness Index; Zonisamide

2018
Metabolic profiling analysis upon acylcarnitines in tissues of hepatocellular carcinoma revealed the inhibited carnitine shuttle system caused by the downregulated carnitine palmitoyltransferase 2.
    Molecular carcinogenesis, 2019, Volume: 58, Issue:5

    Topics: Apoptosis; Biomarkers, Tumor; Carcinoma, Hepatocellular; Carnitine; Carnitine O-Palmitoyltransferase; Case-Control Studies; Cell Proliferation; Follow-Up Studies; Humans; Liver Neoplasms; Metabolome; Prognosis; RNA, Small Interfering; Tumor Cells, Cultured

2019
Frontline Science: Monocytes sequentially rewire metabolism and bioenergetics during an acute inflammatory response.
    Journal of leukocyte biology, 2019, Volume: 105, Issue:2

    Topics: Amino Acids; Carbohydrate Metabolism; Carnitine; Energy Metabolism; Humans; Inflammation; Lipid Metabolism; Lipopolysaccharides; Metabolome; Monocytes; Nucleotides; Principal Component Analysis; THP-1 Cells

2019
Isotope Labeling Strategies for Acylcarnitines Profile in Biological Samples by Liquid Chromatography-Mass Spectrometry.
    Analytical chemistry, 2019, 02-05, Volume: 91, Issue:3

    Topics: Biomarkers; Butylamines; Carnitine; Chromatography, Liquid; Deuterium; Humans; Isotope Labeling; Pyridinium Compounds; Tandem Mass Spectrometry

2019
Characterizing acyl-carnitine biosignatures for schizophrenia: a longitudinal pre- and post-treatment study.
    Translational psychiatry, 2019, 01-17, Volume: 9, Issue:1

    Topics: Adult; Biomarkers; Carnitine; Case-Control Studies; China; Chromatography, Liquid; Energy Metabolism; Female; Humans; Logistic Models; Male; Middle Aged; Multivariate Analysis; Schizophrenia; Tandem Mass Spectrometry

2019
Clinical and muscle magnetic resonance image findings in patients with late-onset multiple acyl-CoA dehydrogenase deficiency.
    Chinese medical journal, 2019, Feb-05, Volume: 132, Issue:3

    Topics: Adolescent; Adult; Biopsy; Carnitine; Electron-Transferring Flavoproteins; Female; Hamstring Muscles; Humans; Iron-Sulfur Proteins; Magnetic Resonance Imaging; Male; Middle Aged; Multiple Acyl Coenzyme A Dehydrogenase Deficiency; Muscle, Skeletal; Oxidoreductases Acting on CH-NH Group Donors; Retrospective Studies; Young Adult

2019
Metabolomic Profiling of Aqueous Humor in Glaucoma Points to Taurine and Spermine Deficiency: Findings from the Eye-D Study.
    Journal of proteome research, 2019, 03-01, Volume: 18, Issue:3

    Topics: Aged; Aged, 80 and over; Amino Acids; Aqueous Humor; Carnitine; Cataract Extraction; Female; Glaucoma, Open-Angle; Humans; Hydroxyproline; Male; Metabolomics; Middle Aged; Spermine; Taurine

2019
Pilot study of myocardial ischemia-induced metabolomic changes in emergency department patients undergoing stress testing.
    PloS one, 2019, Volume: 14, Issue:2

    Topics: Acute Coronary Syndrome; Adult; Aged; Amino Acids; Carnitine; Emergency Service, Hospital; Exercise Test; Female; Humans; Male; Middle Aged; Pilot Projects; Prospective Studies

2019
Age-Specific Cut-off Values of Amino Acids and Acylcarnitines for Diagnosis of Inborn Errors of Metabolism Using Liquid Chromatography Tandem Mass Spectrometry.
    BioMed research international, 2019, Volume: 2019

    Topics: Adolescent; Age Factors; Amino Acids; Carnitine; Child; Child, Preschool; Chromatography, Liquid; Female; Humans; Infant; Infant, Newborn; Male; Metabolism, Inborn Errors; Tandem Mass Spectrometry

2019
Amino acid and fatty acid metabolomic profile during fasting and hyperinsulinemia in girls with polycystic ovarian syndrome.
    American journal of physiology. Endocrinology and metabolism, 2019, 05-01, Volume: 316, Issue:5

    Topics: Absorptiometry, Photon; Adipose Tissue; Adolescent; Amino Acids; Blood Glucose; Body Composition; Calorimetry, Indirect; Carnitine; Case-Control Studies; Child; Estradiol; Fasting; Fatty Acids; Female; Glucose Clamp Technique; Humans; Hyperinsulinism; Insulin Resistance; Liver; Metabolome; Metabolomics; Mitochondria, Muscle; Muscle, Skeletal; Obesity; Polycystic Ovary Syndrome; Sedentary Behavior; Sex Hormone-Binding Globulin; Testosterone

2019
Metabolomic profiling reveals correlations between spermiogram parameters and the metabolites present in human spermatozoa and seminal plasma.
    PloS one, 2019, Volume: 14, Issue:2

    Topics: Adult; Amino Acids; Biogenic Amines; Carnitine; Centrifugation, Density Gradient; Gas Chromatography-Mass Spectrometry; Humans; Lysophosphatidylcholines; Male; Metabolome; Phosphatidylcholines; Semen; Semen Analysis; Spermatozoa; Sphingomyelins; Sugars

2019
Serum carnitine levels of children with epilepsy: Related factors including valproate.
    Brain & development, 2019, Volume: 41, Issue:6

    Topics: Adolescent; Body Mass Index; Carnitine; Child; Child, Preschool; Cognitive Dysfunction; Epilepsy; Female; Humans; Infant; Male; Retrospective Studies; Valproic Acid; Young Adult

2019
Berberine alleviates nonalcoholic fatty liver induced by a high-fat diet in mice by activating SIRT3.
    FASEB journal : official publication of the Federation of American Societies for Experimental Biology, 2019, Volume: 33, Issue:6

    Topics: Acetylation; Acyl-CoA Dehydrogenase, Long-Chain; Animals; Berberine; Carnitine; Diet, High-Fat; Drug Evaluation, Preclinical; Enzyme Activation; Fatty Acids; Glucose; Insulin Resistance; Lipid Metabolism; Male; Metabolome; Mice; Mice, Inbred C57BL; Mice, Knockout; Mitochondria, Liver; Non-alcoholic Fatty Liver Disease; Obesity; Oxidation-Reduction; Protein Processing, Post-Translational; Sirtuin 3

2019
Subchronic low-dose 2,4-D exposure changed plasma acylcarnitine levels and induced gut microbiome perturbations in mice.
    Scientific reports, 2019, 03-13, Volume: 9, Issue:1

    Topics: 2,4-Dichlorophenoxyacetic Acid; Animals; Carnitine; Computational Biology; Environmental Exposure; Gastrointestinal Microbiome; Herbicides; Metabolome; Metabolomics; Metagenome; Mice

2019
Integrated Lipidomics and Metabolomics Analysis of Tears in Multiple Sclerosis: An Insight into Diagnostic Potential of Lacrimal Fluid.
    International journal of molecular sciences, 2019, Mar-13, Volume: 20, Issue:6

    Topics: Adult; Amino Acids; Biomarkers; Carnitine; Central Nervous System; Chromatography, Liquid; Female; Humans; Lacrimal Apparatus; Lipids; Metabolomics; Middle Aged; Multiple Sclerosis; Phospholipids; Tandem Mass Spectrometry; Tears

2019
Peripheral blood metabolite profiles associated with new onset atrial fibrillation.
    American heart journal, 2019, Volume: 211

    Topics: Aged; Amino Acids; Atrial Fibrillation; Biomarkers; Carbohydrates; Carnitine; Coronary Angiography; Fatty Acids; Female; Follow-Up Studies; Humans; Male; Middle Aged; Mitochondria, Heart; Principal Component Analysis; Proportional Hazards Models; Risk Factors

2019
Metabolite aberrations in early diabetes detected in rat kidney using mass spectrometry imaging.
    Analytical and bioanalytical chemistry, 2019, Volume: 411, Issue:13

    Topics: Amino Acids, Branched-Chain; Animals; Carnitine; Diabetes Mellitus, Experimental; Diabetic Nephropathies; Diglycerides; Fatty Acids; Kidney; Lipids; Male; Monoglycerides; Oxidation-Reduction; Rats; Rats, Sprague-Dawley; Spectrometry, Mass, Electrospray Ionization

2019
Plasma acylcarnitines and progression of carotid artery atherosclerosis in HIV infection.
    AIDS (London, England), 2019, 05-01, Volume: 33, Issue:6

    Topics: Adult; Atherosclerosis; Carnitine; Carotid Arteries; Carotid Artery Diseases; Female; HIV Infections; Humans; Longitudinal Studies; Male; Middle Aged; Plasma; Prospective Studies; Risk Factors; Ultrasonography

2019
Implementation of a fast method for the measurement of carnitine palmitoyltransferase 2 activity in lymphocytes by tandem mass spectrometry as confirmation for newborn screening.
    Journal of inherited metabolic disease, 2019, Volume: 42, Issue:5

    Topics: Carnitine; Carnitine O-Palmitoyltransferase; Case-Control Studies; Humans; Infant, Newborn; Lymphocytes; Metabolism, Inborn Errors; Mutation; Neonatal Screening; Tandem Mass Spectrometry

2019
Plasma metabolomic profiling of amino acids and polar lipids in Iranian obese adults.
    Lipids in health and disease, 2019, Apr-09, Volume: 18, Issue:1

    Topics: Adult; Alcohol Drinking; Amino Acids; Biomarkers; Body Mass Index; Carnitine; Case-Control Studies; Chromatography, Liquid; Exercise; Female; Humans; Iran; Linear Models; Lysophospholipids; Male; Metabolome; Metabolomics; Obesity; Phosphatidylcholines; Smoking; Sphingomyelins; Tandem Mass Spectrometry

2019
Western diet consumption through early life induces microvesicular hepatic steatosis in association with an altered metabolome in low birth weight Guinea pigs.
    The Journal of nutritional biochemistry, 2019, Volume: 67

    Topics: Animals; Animals, Newborn; Birth Weight; Carnitine; Diet, Western; Fatty Acids; Female; Guinea Pigs; Lipogenesis; Liver; Metabolome; Non-alcoholic Fatty Liver Disease; Placental Insufficiency; Pregnancy; Weight Gain

2019
Flavin adenine dinucleotide synthase deficiency due to FLAD1 mutation presenting as multiple acyl-CoA dehydrogenation deficiency-like disease: A case report.
    Brain & development, 2019, Volume: 41, Issue:7

    Topics: Acyl Coenzyme A; Carnitine; Child, Preschool; Codon, Nonsense; Electron-Transferring Flavoproteins; Flavin-Adenine Dinucleotide; Humans; Male; Membrane Transport Proteins; Multiple Acyl Coenzyme A Dehydrogenase Deficiency; Mutation; Receptors, G-Protein-Coupled; Riboflavin

2019
Acylcarnitine profile in neonatal hypoxic-ischemic encephalopathy: The value of butyrylcarnitine as a prognostic marker.
    Medicine, 2019, Volume: 98, Issue:15

    Topics: Biomarkers; Brain; Carnitine; Female; Humans; Hypoxia-Ischemia, Brain; Infant, Newborn; Male; Phosphopyruvate Hydratase; Prognosis; Retrospective Studies

2019
A nationwide retrospective observational study of population newborn screening for medium-chain acyl-CoA dehydrogenase (MCAD) deficiency in the Netherlands.
    Journal of inherited metabolic disease, 2019, Volume: 42, Issue:5

    Topics: Acyl-CoA Dehydrogenase; Carnitine; Female; Genotype; Humans; Infant, Newborn; Lipid Metabolism, Inborn Errors; Male; Neonatal Screening; Netherlands; Prevalence; Retrospective Studies

2019
The Impact of OMEGA-3 Fatty Acids Supplementation on Insulin Resistance and Content of Adipocytokines and Biologically Active Lipids in Adipose Tissue of High-Fat Diet Fed Rats.
    Nutrients, 2019, Apr-12, Volume: 11, Issue:4

    Topics: Adiponectin; Adipose Tissue; Animals; Blood Glucose; Carnitine; Carnitine O-Palmitoyltransferase; Ceramides; Diet, High-Fat; Dietary Supplements; Diglycerides; Enzyme-Linked Immunosorbent Assay; Fatty Acids, Omega-3; Fish Oils; Insulin; Insulin Resistance; Intra-Abdominal Fat; Lipid Metabolism; Male; Obesity; Polymerase Chain Reaction; Random Allocation; Rats, Wistar; Subcutaneous Fat

2019
The association between newborn screening analytes and childhood autism in a Texas Medicaid population, 2010-2012.
    American journal of medical genetics. Part B, Neuropsychiatric genetics : the official publication of the International Society of Psychiatric Genetics, 2019, Volume: 180, Issue:5

    Topics: 17-alpha-Hydroxyprogesterone; Autism Spectrum Disorder; Autistic Disorder; Carnitine; Case-Control Studies; Child, Preschool; Female; Humans; Infant; Infant, Newborn; Male; Medicaid; Neonatal Screening; Pilot Projects; Texas; United States

2019
Effect of Acetyl-L-carnitine Used for Protection of Neonatal Hypoxic-Ischemic Brain Injury on Acute Kidney Changes in Male and Female Rats.
    Neurochemical research, 2019, Volume: 44, Issue:10

    Topics: Acetylcarnitine; Animals; Animals, Newborn; Biological Transport; Brain; Brain Injuries; Carnitine; Female; Hypoxia; Hypoxia-Ischemia, Brain; Kidney; Male; Rats, Sprague-Dawley

2019
SIRT3 controls brown fat thermogenesis by deacetylation regulation of pathways upstream of UCP1.
    Molecular metabolism, 2019, Volume: 25

    Topics: 3-Hydroxyacyl CoA Dehydrogenases; Acetyl-CoA C-Acyltransferase; Acetylation; Adipose Tissue, Brown; Adrenergic beta-3 Receptor Antagonists; Animals; Body Composition; Body Temperature Regulation; Carbon-Carbon Double Bond Isomerases; Carnitine; Enoyl-CoA Hydratase; HEK293 Cells; Humans; Male; Mice; Mice, Knockout; Mitochondria; Mitochondrial Proteins; Models, Animal; Mutagenesis; Oxidative Phosphorylation; Proteomics; Racemases and Epimerases; Sirtuin 3; Thermogenesis; Uncoupling Protein 1

2019
Dynamic modelling of an ACADS genotype in fatty acid oxidation - Application of cellular models for the analysis of common genetic variants.
    PloS one, 2019, Volume: 14, Issue:5

    Topics: Acyl-CoA Dehydrogenase; Carnitine; Cell Line, Tumor; Fatty Acids; Female; Genetic Variation; Genome-Wide Association Study; Genotype; Hepatocytes; Humans; Male; Middle Aged; Mitochondria; Oxidation-Reduction; Phenotype

2019
A comprehensive protocol for multiplatform metabolomics analysis in patient-derived skin fibroblasts.
    Metabolomics : Official journal of the Metabolomic Society, 2019, 05-23, Volume: 15, Issue:6

    Topics: Amino Acids; Carnitine; Cells, Cultured; Chromatography, Liquid; Citric Acid Cycle; Fibroblasts; Gas Chromatography-Mass Spectrometry; Humans; Metabolome; Metabolomics; Skin

2019
Seminal plasma metabolome in relation to semen quality and urinary phthalate metabolites among Chinese adult men.
    Environment international, 2019, Volume: 129

    Topics: Adult; Asian People; Carnitine; Chromatography, Liquid; Cross-Sectional Studies; Diethylhexyl Phthalate; Humans; Male; Metabolome; Phthalic Acids; Semen; Semen Analysis; Spermatozoa; Tandem Mass Spectrometry

2019
External Quality Assessment Program for Newborn Screening of Acylcarnitine in China, 2018.
    Clinical laboratory, 2019, Jun-01, Volume: 65, Issue:6

    Topics: Carnitine; China; Humans; Infant, Newborn; Laboratories; Neonatal Screening; Quality Assurance, Health Care; Quality Control; Reference Values; Reproducibility of Results; Tandem Mass Spectrometry

2019
Associations of Amino Acid and Acylcarnitine Profiles With Incident Hyperuricemia in Middle-Aged and Older Chinese Individuals.
    Arthritis care & research, 2020, Volume: 72, Issue:9

    Topics: Aged; Amino Acids; Carnitine; China; Female; Humans; Hyperuricemia; Incidence; Male; Middle Aged; Risk Factors; Uric Acid

2020
Carnitine palmitoyl transferase 1A deficiency in an adult with recurrent severe steato hepatitis aggravated by high pathologic or physiologic demands: A roller-coaster for internists.
    Clinical and molecular hepatology, 2019, Volume: 25, Issue:4

    Topics: Adult; Anti-Bacterial Agents; Carnitine; Carnitine O-Palmitoyltransferase; Escherichia coli Infections; Humans; Hypoglycemia; Lipid Metabolism, Inborn Errors; Liver; Male; Mutation, Missense; Non-alcoholic Fatty Liver Disease; Recurrence; Severity of Illness Index

2019
Prediction of disease severity in multiple acyl-CoA dehydrogenase deficiency: A retrospective and laboratory cohort study.
    Journal of inherited metabolic disease, 2019, Volume: 42, Issue:5

    Topics: Carnitine; Fatty Acids; Female; Humans; Infant, Newborn; Male; Multiple Acyl Coenzyme A Dehydrogenase Deficiency; Retrospective Studies; Severity of Illness Index

2019
Lipid Emulsion Containing High Amounts of n3 Fatty Acids (Omegaven) as Opposed to n6 Fatty Acids (Intralipid) Preserves Insulin Signaling and Glucose Uptake in Perfused Rat Hearts.
    Anesthesia and analgesia, 2020, Volume: 130, Issue:1

    Topics: Animals; Carnitine; Emulsions; Energy Metabolism; Fat Emulsions, Intravenous; Fish Oils; Glucose; Insulin; Isolated Heart Preparation; Myocytes, Cardiac; NF-kappa B; Oxidation-Reduction; Phosphofructokinase-1; Phosphofructokinase-2; Phospholipids; Phosphorylation; Protein Phosphatase 2; Proto-Oncogene Proteins c-akt; Rats, Sprague-Dawley; Signal Transduction; Soybean Oil; Triglycerides

2020
Comprehensive Metabolomic Analysis of IDH1
    Scientific reports, 2019, 07-05, Volume: 9, Issue:1

    Topics: Adult; Aged; Biomarkers, Tumor; Brain Neoplasms; Carnitine; Cell Division; Cell Line, Tumor; Female; Glioblastoma; Glutarates; Humans; Isocitrate Dehydrogenase; Male; Metabolomics; Middle Aged; Mutation; Oxidation-Reduction; Prognosis; Signal Transduction; Transfection

2019
Changes in pediatric plasma acylcarnitines upon fasting for refined interpretation of metabolic stress.
    Molecular genetics and metabolism, 2019, Volume: 127, Issue:4

    Topics: Blood Glucose; Carnitine; Child; Child, Preschool; Fasting; Female; Homeostasis; Humans; Hypoglycemia; Infant; Lipid Metabolism, Inborn Errors; Male; Retrospective Studies; Stress, Physiological

2019
Hydroxylated Long-Chain Acylcarnitines are Biomarkers of Mitochondrial Myopathy.
    The Journal of clinical endocrinology and metabolism, 2019, 12-01, Volume: 104, Issue:12

    Topics: Acyl Coenzyme A; Acyl-CoA Dehydrogenase; Adult; Biomarkers; Carnitine; Cohort Studies; Denmark; Female; Humans; Hydroxylation; Male; Middle Aged; Mitochondrial Myopathies; Mixed Function Oxygenases; Oxidation-Reduction; Prospective Studies

2019
Metabolomic Studies of Tissue Injury in Nonhuman Primates Exposed to Gamma-Radiation.
    International journal of molecular sciences, 2019, Jul-09, Volume: 20, Issue:13

    Topics: Animals; Biomarkers; Carnitine; Female; Gamma Rays; Gene Expression; In Vitro Techniques; Kidney; Lipidomics; Macaca mulatta; Male; Metabolomics; Primates; Radiation, Ionizing

2019
Inter-generational link of obesity in term and preterm births: role of maternal plasma acylcarnitines.
    International journal of obesity (2005), 2019, Volume: 43, Issue:10

    Topics: Adolescent; Adult; Biomarkers; Body Mass Index; Boston; Carnitine; Child; Child, Preschool; Female; Humans; Infant; Infant, Newborn; Male; Mothers; Obesity; Pediatric Obesity; Pregnancy; Premature Birth; Prenatal Exposure Delayed Effects; Prospective Studies

2019
Altered serum acylcarnitine profile is associated with the status of nonalcoholic fatty liver disease (NAFLD) and NAFLD-related hepatocellular carcinoma.
    Scientific reports, 2019, 07-23, Volume: 9, Issue:1

    Topics: Adult; Age Factors; Aged; Carcinoma, Hepatocellular; Carnitine; Disease Progression; Female; Humans; Liver; Liver Cirrhosis; Liver Neoplasms; Male; Middle Aged; Non-alcoholic Fatty Liver Disease

2019
Tryptophan 224 of the rat mitochondrial carnitine/acylcarnitine carrier is crucial for the antiport mechanism.
    Biochimica et biophysica acta. Bioenergetics, 2019, 09-01, Volume: 1860, Issue:9

    Topics: Amino Acid Sequence; Animals; Antiporters; Aspergillus nidulans; Biological Transport; Carnitine; Carnitine Acyltransferases; Models, Molecular; Mutagenesis, Site-Directed; Mutant Proteins; Mutation; Protein Conformation; Rats; Recombinant Proteins; Sequence Homology; Tryptophan

2019
Lipidomics unveils lipid dyshomeostasis and low circulating plasmalogens as biomarkers in a monogenic mitochondrial disorder.
    JCI insight, 2019, 07-25, Volume: 4, Issue:14

    Topics: Adolescent; Animals; Bile Acids and Salts; Biomarkers; Carnitine; Case-Control Studies; Disease Models, Animal; Female; Humans; Leigh Disease; Lipid Metabolism; Lipidomics; Liver; Male; Mice; Mice, Knockout; Mitochondria; Mutation; Neoplasm Proteins; Peroxisomes; Plasmalogens; Prospective Studies; Young Adult

2019
Novel Markers of the Metabolic Impact of Exogenous Retinoic Acid with A Focus on Acylcarnitines and Amino Acids.
    International journal of molecular sciences, 2019, Jul-25, Volume: 20, Issue:15

    Topics: Adipose Tissue; Amino Acids; Animals; Carnitine; Gene Expression Profiling; Leukocytes, Mononuclear; Lipid Metabolism; Lipids; Male; Metabolome; Metabolomics; Mice; Models, Biological; Oxidation-Reduction; Tretinoin

2019
Fatty Acid Metabolites Combine with Reduced β Oxidation to Activate Th17 Inflammation in Human Type 2 Diabetes.
    Cell metabolism, 2019, 09-03, Volume: 30, Issue:3

    Topics: Adult; Aged; Carnitine; Carnitine O-Palmitoyltransferase; Cells, Cultured; Cross-Sectional Studies; Cytokines; Diabetes Mellitus, Type 2; Fatty Acids; Female; Gene Knockdown Techniques; Glycolysis; Humans; Inflammation; Lymphocyte Activation; Male; Membrane Transport Proteins; Middle Aged; Obesity; Oxidation-Reduction; Th17 Cells; Transfection; Young Adult

2019
FLAD1-associated multiple acyl-CoA dehydrogenase deficiency identified by newborn screening.
    Molecular genetics & genomic medicine, 2019, Volume: 7, Issue:9

    Topics: Carnitine; Female; Humans; Infant, Newborn; Multiple Acyl Coenzyme A Dehydrogenase Deficiency; Mutation; Nucleotidyltransferases; Riboflavin

2019
Merged Targeted Quantification and Untargeted Profiling for Comprehensive Assessment of Acylcarnitine and Amino Acid Metabolism.
    Analytical chemistry, 2019, 09-17, Volume: 91, Issue:18

    Topics: Amino Acids; Brain; Brain Chemistry; Calibration; Carnitine; Chromatography, Liquid; Humans; Hydrophobic and Hydrophilic Interactions; Isotope Labeling; Limit of Detection; Mass Spectrometry; Metabolome; Reproducibility of Results

2019
Matrix-Assisted Laser Desorption/Ionization-Mass Spectrometry Imaging of Lipids in Experimental Model of Traumatic Brain Injury Detecting Acylcarnitines as Injury Related Markers.
    Analytical chemistry, 2019, 09-17, Volume: 91, Issue:18

    Topics: Animals; Biomarkers; Brain; Brain Injuries, Traumatic; Carnitine; Disease Models, Animal; Image Processing, Computer-Assisted; Lipid Metabolism; Lipids; Male; Microglia; Palmitoylcarnitine; Rats, Sprague-Dawley; Spectrometry, Mass, Matrix-Assisted Laser Desorption-Ionization; Tandem Mass Spectrometry

2019
Acylcarnitine profiling by low-resolution LC-MS.
    PloS one, 2019, Volume: 14, Issue:8

    Topics: Animals; Carnitine; Chromatography, Liquid; Mass Spectrometry; Mice

2019
Mass Spectrometry Fingerprints of Small-Molecule Metabolites in Biofluids: Building a Spectral Library of Recurrent Spectra for Urine Analysis.
    Analytical chemistry, 2019, 09-17, Volume: 91, Issue:18

    Topics: Body Fluids; Carnitine; Chromatography, Liquid; Humans; Mass Spectrometry; Neoplasms; Small Molecule Libraries; Software

2019
Effect of high-fat diet on peripheral blood mononuclear cells and adipose tissue in early stages of diet-induced weight gain.
    The British journal of nutrition, 2019, 12-28, Volume: 122, Issue:12

    Topics: Animals; Autophagy; Carnitine; Diet, High-Fat; Homeostasis; Inflammation; Insulin; Insulin Resistance; Leukocytes, Mononuclear; Lipid Metabolism; Liver; Male; Obesity; Rabbits; Subcutaneous Fat; Weight Gain

2019
Diagnostic contribution of metabolic workup for neonatal inherited metabolic disorders in the absence of expanded newborn screening.
    Scientific reports, 2019, Oct-01, Volume: 9, Issue:1

    Topics: Amino Acids; Carnitine; Female; Humans; Infant; Infant, Newborn; Infant, Newborn, Diseases; Intensive Care Units, Neonatal; Male; Maple Syrup Urine Disease; Metabolic Diseases; Neonatal Screening; Paris; Phenylketonurias; Propionic Acidemia

2019
Metabolism of fatty acids and bile acids in plasma is associated with overactive bladder in males: potential biomarkers and targets for novel treatments in a metabolomics analysis.
    International urology and nephrology, 2020, Volume: 52, Issue:2

    Topics: Aged; Biomarkers; Carnitine; Case-Control Studies; Cholic Acid; Erucic Acids; Fatty Acids; Fatty Acids, Monounsaturated; Humans; Male; Metabolomics; Nocturia; Urinary Bladder, Overactive; Urine

2020
Exogenous carnitine application augments transport of fatty acids into mitochondria and stimulates mitochondrial respiration in maize seedlings grown under normal and cold conditions.
    Cryobiology, 2019, Volume: 91

    Topics: Animals; Biological Transport; Carnitine; Carnitine O-Palmitoyltransferase; Citrate (si)-Synthase; Cold Temperature; Cold-Shock Response; Cryopreservation; Electron Transport Complex IV; Fatty Acids; Laurates; Mitochondria; Oxidation-Reduction; Seedlings; Zea mays

2019
Severe clinical manifestation of mitochondrial 3-hydroxy-3-methylglutaryl-CoA synthase deficiency associated with two novel mutations: a case report.
    BMC pediatrics, 2019, 10-09, Volume: 19, Issue:1

    Topics: Acidosis; Acyl Coenzyme A; Adipates; Carnitine; Diarrhea; Dicarboxylic Acids; Exome Sequencing; Fatal Outcome; Frameshift Mutation; Glutarates; Humans; Hydroxymethylglutaryl-CoA Synthase; Infant; Male; Mitochondria; Multiple Organ Failure; Mutation; Respiratory Tract Infections

2019
Associations with metabolites in Chinese suggest new metabolic roles in Alzheimer's and Parkinson's diseases.
    Human molecular genetics, 2020, 01-15, Volume: 29, Issue:2

    Topics: Alzheimer Disease; Asian People; ATP-Binding Cassette Transporters; Carnitine; China; DNA-Binding Proteins; Female; Galactosyltransferases; Genetic Loci; Genetic Predisposition to Disease; Genome-Wide Association Study; Glutathione Transferase; Glycosphingolipids; Humans; Hydroxymethylglutaryl-CoA Reductase Inhibitors; Liver-Specific Organic Anion Transporter 1; Lysophospholipids; Male; Membrane Proteins; Middle Aged; Parkinson Disease; Phosphoric Monoester Hydrolases; Serine; Serine C-Palmitoyltransferase; Sphingolipids; Sphingosine; Tandem Mass Spectrometry; Transcription Factors

2020
Clinical and lifestyle related factors influencing whole blood metabolite levels - A comparative analysis of three large cohorts.
    Molecular metabolism, 2019, Volume: 29

    Topics: Adult; Aged; Amino Acids; Carnitine; Chromatography, High Pressure Liquid; Cohort Studies; Coronary Artery Disease; Female; Humans; Life Style; Linear Models; Male; Metabolic Networks and Pathways; Metabolome; Middle Aged; Tandem Mass Spectrometry

2019
MET Inhibition Elicits PGC1α-Dependent Metabolic Reprogramming in Glioblastoma.
    Cancer research, 2020, 01-01, Volume: 80, Issue:1

    Topics: Animals; Antineoplastic Combined Chemotherapy Protocols; Brain Neoplasms; Carnitine; Cell Line, Tumor; Cell Proliferation; Cell Respiration; Crizotinib; Drug Synergism; Epoxy Compounds; Fatty Acids; Gene Expression Profiling; Glioblastoma; Glycolysis; Guanidines; Humans; Lactams, Macrocyclic; Metabolomics; Mice; Mitochondria; Mitochondrial Dynamics; Oxidative Phosphorylation; Peroxisome Proliferator-Activated Receptor Gamma Coactivator 1-alpha; Proteomics; Proto-Oncogene Proteins c-met; Reactive Oxygen Species; Xenograft Model Antitumor Assays

2020
Knockout of the non-essential gene SUGCT creates diet-linked, age-related microbiome disbalance with a diabetes-like metabolic syndrome phenotype.
    Cellular and molecular life sciences : CMLS, 2020, Volume: 77, Issue:17

    Topics: Aging; Animals; Anti-Bacterial Agents; Bacteria; Carnitine; Coenzyme A-Transferases; Dietary Supplements; Feces; Gastrointestinal Microbiome; Humans; Kidney; Lipid Metabolism; Liver; Lysine; Metabolic Syndrome; Metabolome; Mice; Mice, Knockout; Obesity; Tryptophan

2020
The Use of DMS-MS for the Quantitative Analysis of Acylcarnitines.
    Methods in molecular biology (Clifton, N.J.), 2020, Volume: 2084

    Topics: Carnitine; Humans; Ion Mobility Spectrometry; Mass Spectrometry

2020
A Prospective Repurposing of Dantrolene as a Multitarget Agent for Alzheimer's Disease.
    Molecules (Basel, Switzerland), 2019, Nov-25, Volume: 24, Issue:23

    Topics: Acetylcholinesterase; Alzheimer Disease; Calcium; Calcium Channel Blockers; Carnitine; Cell Line; Cholinesterase Inhibitors; Dantrolene; Drug Repositioning; Humans; Malignant Hyperthermia; Monoamine Oxidase; Monoamine Oxidase Inhibitors; Neuroprotective Agents

2019
Systemic and central nervous system metabolic alterations in Alzheimer's disease.
    Alzheimer's research & therapy, 2019, 11-28, Volume: 11, Issue:1

    Topics: Aged; Aged, 80 and over; Alzheimer Disease; Amyloid beta-Peptides; Biomarkers; Brain; Carnitine; Case-Control Studies; Energy Metabolism; Female; Humans; Male; Metabolomics; Middle Aged; Phosphorylation; tau Proteins; Tryptophan

2019
Adolescent Hyperuricemia with Lipid Storage Myopathy: A Clinical Study.
    Medical science monitor : international medical journal of experimental and clinical research, 2019, Nov-30, Volume: 25

    Topics: Adolescent; Adult; Carnitine; Child; China; Electron-Transferring Flavoproteins; Female; Humans; Hyperuricemia; Iron-Sulfur Proteins; Lipid Metabolism, Inborn Errors; Male; Muscle Weakness; Muscle, Skeletal; Muscular Diseases; Muscular Dystrophies; Oxidoreductases Acting on CH-NH Group Donors; Quality of Life; Riboflavin; Young Adult

2019
Brain and blood metabolome for Alzheimer's dementia: findings from a targeted metabolomics analysis.
    Neurobiology of aging, 2020, Volume: 86

    Topics: Aged; Aged, 80 and over; Alzheimer Disease; Biomarkers; Brain; Carnitine; Cognition; Cohort Studies; Female; Follow-Up Studies; Humans; Longitudinal Studies; Male; Metabolome; Metabolomics; Middle Aged; Risk

2020
One potential hotspot ACADVL mutation in Chinese patients with very-long-chain acyl-coenzyme A dehydrogenase deficiency.
    Clinica chimica acta; international journal of clinical chemistry, 2020, Volume: 503

    Topics: Acyl-CoA Dehydrogenase, Long-Chain; Asian People; Carnitine; China; Congenital Bone Marrow Failure Syndromes; Female; Humans; Hypoglycemia; Infant, Newborn; Lipid Metabolism, Inborn Errors; Liver Diseases; Male; Mitochondrial Diseases; Muscular Diseases; Mutation

2020
Homozygous B4GALNT1 mutation and biochemical glutaric acidemia type II: A case report.
    Clinical neurology and neurosurgery, 2020, Volume: 189

    Topics: Adult; Carnitine; Epilepsy, Temporal Lobe; Female; Frameshift Mutation; Homozygote; Humans; Intellectual Disability; Multiple Acyl Coenzyme A Dehydrogenase Deficiency; N-Acetylgalactosaminyltransferases; Spastic Paraplegia, Hereditary

2020
Plasma metabolomics in tuberculosis patients with and without concurrent type 2 diabetes at diagnosis and during antibiotic treatment.
    Scientific reports, 2019, 12-10, Volume: 9, Issue:1

    Topics: Adult; Amines; Anti-Bacterial Agents; Blood Proteins; Body Weight; Carnitine; Chromatography, Liquid; Diabetes Mellitus, Type 2; Female; Humans; Indoleamine-Pyrrole 2,3,-Dioxygenase; Indonesia; Least-Squares Analysis; Male; Metabolomics; Middle Aged; Nitric Oxide Synthase; Principal Component Analysis; ROC Curve; Tandem Mass Spectrometry; Tuberculosis, Pulmonary; Young Adult

2019
Preoperative plasma fatty acid metabolites inform risk of prostate cancer progression and may be used for personalized patient stratification.
    BMC cancer, 2019, Dec-16, Volume: 19, Issue:1

    Topics: Aged; Carnitine; Case-Control Studies; Chromatography, Liquid; Disease Progression; Fatty Acids; Humans; Male; Mass Spectrometry; Metabolomics; Middle Aged; Prognosis; Prostatic Neoplasms; White People

2019
Slc22a5 haploinsufficiency does not aggravate the phenotype of the long-chain acyl-CoA dehydrogenase KO mouse.
    Journal of inherited metabolic disease, 2020, Volume: 43, Issue:3

    Topics: Acyl-CoA Dehydrogenase, Long-Chain; Animals; Cardiomyopathies; Carnitine; Disease Models, Animal; Fatty Acids; Female; Haploinsufficiency; Hyperammonemia; Lipid Metabolism; Liver; Male; Mice; Mice, Knockout; Muscular Diseases; Myocardium; Phenotype; Solute Carrier Family 22 Member 5

2020
Metabolic and lipidomic characterization of malignant pleural effusion in human lung cancer.
    Journal of pharmaceutical and biomedical analysis, 2020, Feb-20, Volume: 180

    Topics: Adenocarcinoma; Biomarkers, Tumor; Carnitine; Chromatography, Liquid; Down-Regulation; Fatty Acids, Unsaturated; Female; Humans; Lipid Metabolism; Lipidomics; Lung Neoplasms; Male; Mass Spectrometry; Metabolome; Metabolomics; Middle Aged; Multivariate Analysis; Pleural Effusion, Malignant; Up-Regulation

2020
Stimulating pyruvate dehydrogenase complex reduces itaconate levels and enhances TCA cycle anabolic bioenergetics in acutely inflamed monocytes.
    Journal of leukocyte biology, 2020, Volume: 107, Issue:3

    Topics: Algorithms; Amino Acids; Carnitine; Citric Acid Cycle; Cytokines; Energy Metabolism; Humans; Inflammation; Ketones; Lipopolysaccharides; Metabolome; Monocytes; Principal Component Analysis; Pyruvate Dehydrogenase Complex; Succinates; THP-1 Cells

2020
Genetic deletion of gpr27 alters acylcarnitine metabolism, insulin sensitivity, and glucose homeostasis in zebrafish.
    FASEB journal : official publication of the Federation of American Societies for Experimental Biology, 2020, Volume: 34, Issue:1

    Topics: Animals; Carnitine; Carnitine O-Palmitoyltransferase; Gene Deletion; Glucose; Homeostasis; Insulin; Insulin Resistance; Proto-Oncogene Proteins c-akt; Receptors, G-Protein-Coupled; Signal Transduction; Zebrafish

2020
Loss of function in the Drosophila clock gene period results in altered intermediary lipid metabolism and increased susceptibility to starvation.
    Cellular and molecular life sciences : CMLS, 2020, Volume: 77, Issue:23

    Topics: Animals; Biomarkers; Carnitine; Circadian Clocks; Circadian Rhythm; Drosophila melanogaster; Drosophila Proteins; Feeding Behavior; Lipid Metabolism; Lipids; Loss of Function Mutation; Male; Metabolome; Motor Activity; Period Circadian Proteins; Starvation; Stress, Physiological; Tryptophan

2020
Potential osteomyelitis biomarkers identified by plasma metabolome analysis in mice.
    Scientific reports, 2020, 01-21, Volume: 10, Issue:1

    Topics: Animals; Biomarkers; Carnitine; Citric Acid Cycle; Disease Models, Animal; Fatty Acids; Ketoglutaric Acids; Luminescent Measurements; Male; Metabolome; Mice, Inbred BALB C; Osteomyelitis; Sphingolipids; Sphingosine; Staphylococcal Infections; Staphylococcus aureus; Succinic Acid; Surgical Wound Infection

2020
Evaluation of a panel of very long-chain lysophosphatidylcholines and acylcarnitines for screening of X-linked adrenoleukodystrophy in China.
    Clinica chimica acta; international journal of clinical chemistry, 2020, Volume: 503

    Topics: Adrenoleukodystrophy; Age Factors; Biomarkers; Carnitine; Case-Control Studies; Child; Dried Blood Spot Testing; Female; Genetic Diseases, X-Linked; Humans; Infant, Newborn; Lysophosphatidylcholines; Male; Mass Screening; Tandem Mass Spectrometry

2020
Serum acylcarnitine profile in endurance horses with and without metabolic dysfunction.
    Veterinary journal (London, England : 1997), 2020, Volume: 255

    Topics: Animals; Carnitine; Fatty Acids; Female; Horses; Male; Metabolic Diseases; Mitochondria; Oxidation-Reduction; Physical Endurance; Running; Tandem Mass Spectrometry

2020
Quantitation of Urinary Acylcarnitines by DMS-MS/MS Uncovers the Effects of Total Body Irradiation in Cancer Patients.
    Journal of the American Society for Mass Spectrometry, 2020, Mar-04, Volume: 31, Issue:3

    Topics: Animals; Biomarkers; Carnitine; Humans; Neoplasms; Pilot Projects; Rats, Sprague-Dawley; Tandem Mass Spectrometry; Whole-Body Irradiation

2020
Metabolomic Analysis of the Liver of a Dextran Sodium Sulfate-Induced Acute Colitis Mouse Model: Implications of the Gut-Liver Connection.
    Cells, 2020, 02-01, Volume: 9, Issue:2

    Topics: Acute Disease; Animals; Body Weight; Carnitine; Colitis; Colon; Discriminant Analysis; Disease Models, Animal; Gastrointestinal Tract; Least-Squares Analysis; Liver; Male; Metabolome; Metabolomics; Mice, Inbred BALB C; Multivariate Analysis; Proton Magnetic Resonance Spectroscopy; Rats; Sulfates

2020
Acylcarnitines at the Membrane Surface: Insertion Parameters for a Mitochondrial Leaflet Model.
    Biophysical journal, 2020, 03-10, Volume: 118, Issue:5

    Topics: Carnitine; Membranes; Mitochondrial Membranes; Static Electricity

2020
Exercise with food withdrawal at thermoneutrality impacts fuel use, the microbiome, AMPK phosphorylation, muscle fibers, and thyroid hormone levels in rats.
    Physiological reports, 2020, Volume: 8, Issue:3

    Topics: AMP-Activated Protein Kinase Kinases; Animals; Carnitine; Energy Metabolism; Fasting; Gastrointestinal Microbiome; Ketone Bodies; Lipid Metabolism; Liver; Male; Motor Activity; Muscle Fibers, Skeletal; Phosphorylation; Protein Kinases; Rats; Rats, Wistar; Temperature; Thyroid Hormones

2020
Analysis of amino acids and acylcarnitines profiles in small, appropriate, and large for gestational age neonates.
    The journal of maternal-fetal & neonatal medicine : the official journal of the European Association of Perinatal Medicine, the Federation of Asia and Oceania Perinatal Societies, the International Society of Perinatal Obstetricians, 2022, Volume: 35, Issue:3

    Topics: Amino Acids; Birth Weight; Carnitine; Gestational Age; Humans; Infant, Newborn; Infant, Newborn, Diseases; Infant, Small for Gestational Age

2022
Increased antioxidant response in medium-chain acyl-CoA dehydrogenase deficiency: does lipoic acid have a protective role?
    Pediatric research, 2020, Volume: 88, Issue:4

    Topics: Acyl-CoA Dehydrogenase; Antioxidants; Caprylates; Carnitine; Cell Death; Fibroblasts; Genotype; Glycolysis; Humans; Lipid Metabolism, Inborn Errors; Lipid Peroxidation; Mitochondria; Oxidative Stress; Phenotype; Superoxides; Thioctic Acid

2020
Acylcarnitine metabolomic profiles inform clinically-defined major depressive phenotypes.
    Journal of affective disorders, 2020, 03-01, Volume: 264

    Topics: Carnitine; Depressive Disorder, Major; Humans; Phenotype; Retrospective Studies

2020
Effects of the Menstrual Cycle on Serum Carnitine and Endurance Performance of Women.
    International journal of sports medicine, 2020, Volume: 41, Issue:7

    Topics: Carnitine; Estradiol; Exercise; Exercise Test; Female; Follicular Phase; Humans; Luteal Phase; Physical Endurance; Progesterone; Young Adult

2020
Targeted Metabolomics Analysis Reveals that Dietary Supranutritional Selenium Regulates Sugar and Acylcarnitine Metabolism Homeostasis in Pig Liver.
    The Journal of nutrition, 2020, 04-01, Volume: 150, Issue:4

    Topics: Animals; Carnitine; Diabetes Mellitus, Type 2; Diet; Dietary Supplements; Dose-Response Relationship, Drug; Homeostasis; Hyperglycemia; Hyperinsulinism; Lipids; Liver; Male; Metabolomics; Models, Animal; Oxidation-Reduction; RNA, Messenger; Selenium; Selenomethionine; Selenoproteins; Sugars; Sus scrofa

2020
Prediction of VLCAD deficiency phenotype by a metabolic fingerprint in newborn screening bloodspots.
    Biochimica et biophysica acta. Molecular basis of disease, 2020, 06-01, Volume: 1866, Issue:6

    Topics: Acyl-CoA Dehydrogenase, Long-Chain; Carnitine; Child; Child, Preschool; Congenital Bone Marrow Failure Syndromes; Dried Blood Spot Testing; Female; Humans; Infant; Infant, Newborn; Lipid Metabolism, Inborn Errors; Male; Mass Spectrometry; Metabolomics; Mitochondrial Diseases; Muscular Diseases; Neonatal Screening; Phenotype

2020
Blood carnitine profiling on tandem mass spectrometry in liver cirrhotic patients.
    BMC gastroenterology, 2020, Feb-19, Volume: 20, Issue:1

    Topics: Adult; Aged; Aged, 80 and over; Carnitine; Female; Humans; Liver Cirrhosis; Male; Middle Aged; Surveys and Questionnaires; Tandem Mass Spectrometry

2020
Simultaneous quantification of free fatty acids and acylcarnitines in plasma samples using dansylhydrazine labeling and liquid chromatography-triple quadrupole mass spectrometry.
    Analytical and bioanalytical chemistry, 2020, Volume: 412, Issue:12

    Topics: Carbon Isotopes; Carnitine; Chromatography, Liquid; Dansyl Compounds; Humans; Hydrazines; Isotope Labeling; Tandem Mass Spectrometry

2020
Identification and regulation of the xenometabolite derivatives
    American journal of physiology. Endocrinology and metabolism, 2020, 05-01, Volume: 318, Issue:5

    Topics: Carnitine; Exercise; Humans; Male; Muscle, Skeletal; Young Adult

2020
Neonatal Macrosomia is an Interfering Factor for Analytes on the Colorado State Newborn Screen.
    The Journal of clinical endocrinology and metabolism, 2020, 03-01, Volume: 105, Issue:3

    Topics: Adult; Amino Acid Metabolism, Inborn Errors; Carnitine; Carnitine O-Palmitoyltransferase; Colorado; False Positive Reactions; Female; Fetal Macrosomia; Humans; Infant, Newborn; Infant, Newborn, Diseases; Male; Metabolism, Inborn Errors; Neonatal Screening; Pediatric Obesity; Pregnancy; Propionic Acidemia; Tandem Mass Spectrometry

2020
Rare variants and biological pathways identified in treatment-refractory depression.
    Journal of neuroscience research, 2020, Volume: 98, Issue:7

    Topics: Adolescent; Adult; Alleles; Biopterins; Carnitine; Computer Simulation; Depressive Disorder, Treatment-Resistant; Exome Sequencing; Female; Genetic Predisposition to Disease; Humans; Male; Phenotype; Polymorphism, Single Nucleotide; Tetrahydrofolates; Young Adult

2020
Post-mortem tissue analyses in a patient with succinic semialdehyde dehydrogenase deficiency (SSADHD). I. Metabolomic outcomes.
    Metabolic brain disease, 2020, Volume: 35, Issue:4

    Topics: Adult; Amino Acid Metabolism, Inborn Errors; Amino Acids; Brain; Carnitine; Creatine; Creatinine; Developmental Disabilities; gamma-Aminobutyric Acid; Glycine; Humans; Male; Metabolomics; Succinate-Semialdehyde Dehydrogenase

2020
Metabolic profiling by reversed-phase/ion-exchange mass spectrometry.
    Journal of chromatography. B, Analytical technologies in the biomedical and life sciences, 2020, Apr-15, Volume: 1143

    Topics: Amino Acids; Bile Acids and Salts; Carnitine; Chromatography, High Pressure Liquid; Chromatography, Ion Exchange; Chromatography, Reverse-Phase; Hormones; Humans; Hydrophobic and Hydrophilic Interactions; Isoleucine; Metabolome; Metabolomics; Methylmalonic Acid; Nucleosides; Small Molecule Libraries; Succinic Acid; Tandem Mass Spectrometry; Vitamins

2020
Plasma Metabolomic Markers of Insulin Resistance and Diabetes and Rate of Incident Parkinson's Disease.
    Journal of Parkinson's disease, 2020, Volume: 10, Issue:3

    Topics: Adult; Aged; Biomarkers; Carnitine; Diabetes Mellitus; Female; Humans; Insulin Resistance; Male; Metabolomics; Middle Aged; Parkinson Disease; Plasma; Risk Factors

2020
Carnitine palmitoyltransferase 1C contributes to progressive cellular senescence.
    Aging, 2020, 04-14, Volume: 12, Issue:8

    Topics: Animals; Carcinogenesis; Carcinoma; Carnitine; Carnitine O-Palmitoyltransferase; Cell Line, Tumor; Cell Movement; Cell Proliferation; Cell Survival; Cellular Senescence; Cyclin-Dependent Kinase Inhibitor p21; DNA-Binding Proteins; Gene Expression Regulation; Genetic Vectors; Humans; Male; Metabolomics; Mice; Mitochondria; Mitochondrial Proteins; Mitophagy; Neoplasm Transplantation; Nuclear Respiratory Factor 1; Pancreatic Neoplasms; Peroxisome Proliferator-Activated Receptor Gamma Coactivator 1-alpha; PPAR alpha; Protein Transport; RNA, Messenger; Signal Transduction; Telomere Shortening; Transcription Factors; Tumor Suppressor Proteins

2020
Inborn errors of metabolism detectable by tandem mass spectrometry in Beijing.
    Journal of pediatric endocrinology & metabolism : JPEM, 2020, May-26, Volume: 33, Issue:5

    Topics: Amino Acids; Beijing; Carnitine; Female; Humans; Infant, Newborn; Male; Metabolism, Inborn Errors; Neonatal Screening; Tandem Mass Spectrometry

2020
Relationship between the plasma acylcarnitine profile and cardiometabolic risk factors in adults diagnosed with cardiovascular diseases.
    Clinica chimica acta; international journal of clinical chemistry, 2020, Volume: 507

    Topics: Adult; Aged; Cardiometabolic Risk Factors; Cardiovascular Diseases; Carnitine; Female; Humans; Male; Middle Aged

2020
Contributions of amino acid, acylcarnitine and sphingolipid profiles to type 2 diabetes risk among South-Asian Surinamese and Dutch adults.
    BMJ open diabetes research & care, 2020, Volume: 8, Issue:1

    Topics: Adult; Amino Acids; Carnitine; Diabetes Mellitus, Type 2; Ethnicity; Humans; Netherlands; Sphingolipids

2020
The Association Between Acylcarnitine Metabolites and Cardiovascular Disease in Chinese Patients With Type 2 Diabetes Mellitus.
    Frontiers in endocrinology, 2020, Volume: 11

    Topics: Aged; Biomarkers; Cardiovascular Diseases; Carnitine; Cross-Sectional Studies; Diabetes Mellitus, Type 2; Female; Follow-Up Studies; Humans; Male; Metabolome; Middle Aged; Prognosis

2020
Sexual Dimorphism in Energy Metabolism of Wistar Rats Using Data Analysis.
    Molecules (Basel, Switzerland), 2020, May-18, Volume: 25, Issue:10

    Topics: Amino Acids; Animals; Biomarkers; Carnitine; Data Analysis; Discriminant Analysis; Energy Metabolism; Female; Male; Metabolome; Metabolomics; Phenylalanine; Rats; Sex Characteristics; Tyrosine

2020
Metabolomics of a mouse model of preeclampsia induced by overexpressing soluble fms-like tyrosine kinase 1.
    Biochemical and biophysical research communications, 2020, 07-05, Volume: 527, Issue:4

    Topics: Animals; Carnitine; Cell Line; Disease Models, Animal; Fatty Acids; Female; Fetal Growth Retardation; Glycolysis; Humans; Metabolomics; Mice; Mice, Inbred ICR; Placenta; Pre-Eclampsia; Pregnancy; Up-Regulation; Vascular Endothelial Growth Factor Receptor-1

2020
Determination of carnitine ester profile in the children with type 1 diabetes: a valuable step towards a better management.
    Archives of physiology and biochemistry, 2022, Volume: 128, Issue:5

    Topics: Amino Acids; Carnitine; Child; Coenzyme A; Diabetes Mellitus, Type 1; Esters; Fatty Acids; Female; Glycated Hemoglobin; Humans; Isotopes; Male

2022
Association Between the Serum Carnitine Level and Ammonia and Valproic Acid Levels in Patients with Bipolar Disorder.
    Therapeutic drug monitoring, 2020, Volume: 42, Issue:5

    Topics: Ammonia; Anticonvulsants; Bipolar Disorder; Carnitine; Epilepsy; Female; Humans; Male; Middle Aged; Retrospective Studies; Valproic Acid

2020
Metabolic Effects of Gastric Bypass Surgery: Is It All About Calories?
    Diabetes, 2020, Volume: 69, Issue:9

    Topics: Adult; Blood Glucose; Caloric Restriction; Carnitine; Diabetes Mellitus, Type 2; Fasting; Female; Gastric Bypass; Humans; Insulin; Insulin Resistance; Middle Aged; Obesity, Morbid; Postprandial Period

2020
Targeted Metabolomic Analysis of a Mucopolysaccharidosis IIIB Mouse Model Reveals an Imbalance of Branched-Chain Amino Acid and Fatty Acid Metabolism.
    International journal of molecular sciences, 2020, Jun-12, Volume: 21, Issue:12

    Topics: Amino Acids, Aromatic; Amino Acids, Branched-Chain; Animals; Carnitine; Disease Models, Animal; Humans; Lipid Metabolism; Liver; Male; Metabolomics; Mice; Mucopolysaccharidosis III; Myocardium

2020
Plasma acylcarnitine levels increase with healthy aging.
    Aging, 2020, 06-16, Volume: 12, Issue:13

    Topics: Adult; Age Factors; Aged; Aged, 80 and over; Carnitine; Cross-Sectional Studies; Energy Metabolism; Fasting; Feasibility Studies; Female; Healthy Aging; Healthy Volunteers; Humans; Male; Metabolomics; Middle Aged; Mitochondria; Oxidation-Reduction; Sex Factors; Young Adult

2020
HDAC11 is a novel regulator of fatty acid oxidative metabolism in skeletal muscle.
    The FEBS journal, 2021, Volume: 288, Issue:3

    Topics: AMP-Activated Protein Kinases; Animals; Carnitine; Energy Metabolism; Fatty Acids; Gene Expression Regulation; Glycolysis; Histone Deacetylases; Mice, Inbred C57BL; Mice, Knockout; Mitochondria, Muscle; Muscle Fibers, Skeletal; Muscle, Skeletal; Oxidation-Reduction

2021
Does metabolomic profile differ with regard to birth weight?
    Pediatric research, 2021, Volume: 89, Issue:5

    Topics: Birth Weight; Carnitine; Female; Humans; Iceland; Infant, Newborn; Male; Metabolomics; Neonatal Screening

2021
Intramuscular short-chain acylcarnitines in elderly people are decreased in (pre-)frail females, but not in males.
    FASEB journal : official publication of the Federation of American Societies for Experimental Biology, 2020, Volume: 34, Issue:9

    Topics: Aged; Aged, 80 and over; Aging; Carnitine; Cross-Sectional Studies; Female; Frail Elderly; Frailty; Hand Strength; Humans; Male; Muscles; Physical Fitness; Sex Factors; Walking

2020
Relationship between fermented dairy consumption, circulating short-chain acylcarnitines and angiographic severity of coronary artery disease.
    Nutrition, metabolism, and cardiovascular diseases : NMCD, 2020, 09-24, Volume: 30, Issue:10

    Topics: Aged; Biomarkers; Carnitine; Coronary Angiography; Coronary Artery Disease; Coronary Stenosis; Cross-Sectional Studies; Cultured Milk Products; Female; Germany; Humans; Male; Middle Aged; Predictive Value of Tests; Prevalence; Protective Factors; Risk Factors; Severity of Illness Index

2020
A targeted metabolomic profiling of plasma acylcarnitines in nonalcoholic fatty liver disease.
    European review for medical and pharmacological sciences, 2020, Volume: 24, Issue:13

    Topics: Adult; Biomarkers; Carnitine; Case-Control Studies; Chromatography, High Pressure Liquid; Female; Humans; Male; Metabolome; Metabolomics; Middle Aged; Non-alcoholic Fatty Liver Disease; Spectrometry, Mass, Electrospray Ionization; Tandem Mass Spectrometry

2020
Metabolomic signature of the seminal plasma in men with severe oligoasthenospermia.
    Andrology, 2020, Volume: 8, Issue:6

    Topics: Adult; Amines; Amino Acids; Carnitine; Fatty Acids, Unsaturated; Humans; Male; Metabolome; Metabolomics; Oligospermia; Phosphatidylcholines; Prospective Studies; Semen; Semen Analysis; Sperm Motility; Spermatozoa; Sphingomyelins

2020
Perinatal free carnitine and short chain acylcarnitine blood concentrations in 12,000 full-term breastfed newborns in relation to their birth weight.
    Pediatrics and neonatology, 2020, Volume: 61, Issue:6

    Topics: Biomarkers; Birth Weight; Breast Feeding; Carnitine; Chromatography, Liquid; Fatty Acids; Female; Humans; Infant; Infant, Newborn; Male; Neonatal Screening; Reference Values; Tandem Mass Spectrometry

2020
Quantitative Metabolomics Reveals Heart Failure With Midrange Ejection Fraction as a Distinct Phenotype of Heart Failure.
    The Canadian journal of cardiology, 2021, Volume: 37, Issue:2

    Topics: Amino Acids; Analysis of Variance; Biomarkers; Carnitine; China; Female; Heart Failure; Humans; Kaplan-Meier Estimate; Male; Metabolome; Metabolomics; Middle Aged; Patient Acuity; Phenotype; Predictive Value of Tests; Prognosis; ROC Curve; Stroke Volume

2021
Kinetics of Serum Carnitine Fractions in Patients with Chronic Kidney Disease Not on Dialysis.
    The Kurume medical journal, 2021, Oct-06, Volume: 66, Issue:3

    Topics: Adult; Aged; Amino Acids; Carnitine; Female; Glomerular Filtration Rate; Humans; Kidney; Kidney Failure, Chronic; Kinetics; Male; Middle Aged; Renal Insufficiency, Chronic; Urea; Uric Acid

2021
Decreases in Circulating Concentrations of Short-Chain Acylcarnitines are Associated with Systolic Function Improvement After Decompensated Heart Failure.
    International heart journal, 2020, Sep-29, Volume: 61, Issue:5

    Topics: Aged; Carnitine; Case-Control Studies; Esters; Fatty Acids; Female; Heart Failure; Humans; Male; Mass Spectrometry; Metabolomics; Middle Aged; Prognosis; Stroke Volume; Systole

2020
Distinct lipid signatures are identified in the plasma of rats with chronic inflammation induced by estradiol benzoate and sex hormones.
    Metabolomics : Official journal of the Metabolomic Society, 2020, 09-07, Volume: 16, Issue:9

    Topics: Amino Acids; Animals; Biomarkers; Carnitine; Estradiol; Glycerophospholipids; Glycine; Gonadal Steroid Hormones; Humans; Inflammation; Lipids; Male; Metabolomics; Plasma; Prostatic Neoplasms; Prostatitis; Rats; Rats, Sprague-Dawley; Sphingomyelins

2020
Untargeted lipidomics reveals specific lipid abnormalities in Sjögren's syndrome.
    Rheumatology (Oxford, England), 2021, 03-02, Volume: 60, Issue:3

    Topics: Biomarkers; Carnitine; Case-Control Studies; Chromatography, High Pressure Liquid; Discriminant Analysis; Female; Humans; Lipidomics; Male; Mass Spectrometry; Phosphatidylcholines; Sensitivity and Specificity; Sjogren's Syndrome; Triglycerides

2021
Untargeted metabolomics yields insight into ALS disease mechanisms.
    Journal of neurology, neurosurgery, and psychiatry, 2020, Volume: 91, Issue:12

    Topics: Aged; Amyotrophic Lateral Sclerosis; Benzoates; Carnitine; Case-Control Studies; Ceramides; Creatine; Discriminant Analysis; Fatty Acids; Fatty Acids, Unsaturated; Female; Humans; Least-Squares Analysis; Logistic Models; Machine Learning; Male; Metabolic Networks and Pathways; Metabolomics; Middle Aged

2020
[Recommendations for acylcarnitine profile analysis].
    Annales de biologie clinique, 2020, 10-01, Volume: 78, Issue:5

    Topics: Accreditation; Adult; Amniocentesis; Amniotic Fluid; Blood Chemical Analysis; Blood Specimen Collection; Carnitine; Child; Chromatography, Paper; Clinical Laboratory Services; Diagnostic Tests, Routine; Female; Humans; Infant, Newborn; Male; Metabolism, Inborn Errors; Neonatal Screening; Pre-Analytical Phase; Pregnancy; Prenatal Diagnosis; Urinalysis; Urine Specimen Collection

2020
Proteinuria as a presenting sign of combined methylmalonic acidemia and homocysteinemia: case report.
    BMC medical genetics, 2020, 09-21, Volume: 21, Issue:1

    Topics: Adolescent; Amino Acid Metabolism, Inborn Errors; Amino Acids; Base Sequence; Carnitine; Child, Preschool; DNA; Gas Chromatography-Mass Spectrometry; Homocysteine; Humans; Hyperhomocysteinemia; Male; Methylmalonic Acid; Proteinuria

2020
Acyl-Carnitine plasma levels and their association with metabolic syndrome in individuals with schizophrenia.
    Psychiatry research, 2020, Volume: 293

    Topics: Adult; Biomarkers; Carnitine; Case-Control Studies; Female; Humans; Male; Metabolic Syndrome; Plasma; Schizophrenia; Schizophrenic Psychology

2020
Serum acylcarnitines and amino acids and risk of type 2 diabetes in a multiethnic Asian population.
    BMJ open diabetes research & care, 2020, Volume: 8, Issue:1

    Topics: Amino Acids; Carnitine; Diabetes Mellitus, Type 2; Female; Humans; Male; Prospective Studies; Risk Factors; Singapore

2020
Laboratory analysis of acylcarnitines, 2020 update: a technical standard of the American College of Medical Genetics and Genomics (ACMG).
    Genetics in medicine : official journal of the American College of Medical Genetics, 2021, Volume: 23, Issue:2

    Topics: Carnitine; Genetics, Medical; Genomics; Humans; Infant, Newborn; Laboratories; United States

2021
Testosterone replacement therapy in blood donors modulates erythrocyte metabolism and susceptibility to hemolysis in cold storage.
    Transfusion, 2021, Volume: 61, Issue:1

    Topics: Animals; Arginine; Blood Donors; Blood Preservation; Carnitine; Chromatography, High Pressure Liquid; Cohort Studies; Correlation of Data; Dopamine; Erucic Acids; Erythrocytes; Fatty Acids; Female; Glutathione; Hemolysis; Hormone Replacement Therapy; Humans; Male; Mass Spectrometry; Metabolic Networks and Pathways; Mice; Mice, Inbred Strains; Oxidation-Reduction; Pentose Phosphate Pathway; Purines; Testosterone; Tumor Protein, Translationally-Controlled 1

2021
Altered Acylcarnitine Metabolism Is Associated With an Increased Risk of Atrial Fibrillation.
    Journal of the American Heart Association, 2020, 11-03, Volume: 9, Issue:21

    Topics: Aged; Atrial Fibrillation; Carnitine; Cohort Studies; Female; Humans; Incidence; Male; Metabolomics; Middle Aged; Proportional Hazards Models; Risk Factors

2020
Association of cardiac autonomic dysfunction with higher levels of plasma lipid metabolites in recent-onset type 2 diabetes.
    Diabetologia, 2021, Volume: 64, Issue:2

    Topics: Adult; Autonomic Nervous System Diseases; Carnitine; Diabetes Mellitus, Type 1; Diabetes Mellitus, Type 2; Diabetic Neuropathies; Dyslipidemias; Fatty Acids, Nonesterified; Female; Glucose Clamp Technique; Heart Rate; Humans; Insulin Resistance; Lipid Metabolism; Lipidomics; Lysophosphatidylcholines; Male; Middle Aged; Obesity; Phosphatidylcholines; Sphingomyelins; Young Adult

2021
Liraglutide treatment and acylcarnitine profiles in Egyptian obese insulin-resistant females.
    European journal of pharmacology, 2021, Jan-15, Volume: 891

    Topics: Adult; Anti-Obesity Agents; Biomarkers; Blood Glucose; Carnitine; Case-Control Studies; Egypt; Female; Glycated Hemoglobin; Humans; Hypoglycemic Agents; Insulin Resistance; Liraglutide; Metabolomics; Middle Aged; Mitochondria; Obesity; Time Factors; Treatment Outcome; Weight Loss

2021
Macrophages and acylcarnitines: New players in aspirin-exacerbated respiratory disease?
    The Journal of allergy and clinical immunology, 2021, Volume: 147, Issue:2

    Topics: Aspirin; Asthma, Aspirin-Induced; Carnitine; Humans; Macrophages

2021
Altered Serum Amino Acid and Acylcarnitine Profiles in Hyperinsulinemic Hypoglycemia and Ketotic Hypoglycemia.
    Frontiers in endocrinology, 2020, Volume: 11

    Topics: Amino Acids; Biomarkers; Carnitine; Case-Control Studies; Child, Preschool; Congenital Hyperinsulinism; Female; Follow-Up Studies; Humans; Hypoglycemia; Infant; Ketosis; Male; Prognosis; Retrospective Studies

2020
Mitochondrial dysfunction in inflammatory bowel disease alters intestinal epithelial metabolism of hepatic acylcarnitines.
    The Journal of clinical investigation, 2021, 01-04, Volume: 131, Issue:1

    Topics: Animals; Caco-2 Cells; Carnitine; Citrobacter rodentium; Enterobacteriaceae Infections; Female; Humans; Inflammatory Bowel Diseases; Intestinal Mucosa; Liver; Male; Mice; Mice, Inbred BALB C; Mitochondria

2021
Loss of Muscle Carnitine Palmitoyltransferase 2 Prevents Diet-Induced Obesity and Insulin Resistance despite Long-Chain Acylcarnitine Accumulation.
    Cell reports, 2020, 11-10, Volume: 33, Issue:6

    Topics: Animals; Carnitine; Carnitine O-Palmitoyltransferase; Female; Humans; Insulin Resistance; Mice; Obesity

2020
Plasma metabolomics supports the use of long-duration cardiac arrest rodent model to study human disease by demonstrating similar metabolic alterations.
    Scientific reports, 2020, 11-12, Volume: 10, Issue:1

    Topics: Animals; Biomarkers; Cardiopulmonary Resuscitation; Carnitine; Disease Models, Animal; Fatty Acids; Heart Arrest; Humans; Kynurenic Acid; Male; Metabolomics; Phospholipids; Rats; Rats, Sprague-Dawley; Severity of Illness Index; Tryptophan

2020
Novel metabolomics markers are associated with pre-clinical decline in hand grip strength in community-dwelling older adults.
    Mechanisms of ageing and development, 2021, Volume: 193

    Topics: Aged; Aging; Asymptomatic Diseases; Biomarkers; Carnitine; Energy Metabolism; Fatty Acids; Female; Frailty; Geriatric Assessment; Hand Strength; Humans; Independent Living; Longitudinal Studies; Male; Metabolomics; Mitochondria, Muscle; Prognosis; Sarcopenia

2021
Enhancing confidence of metabolite annotation in Capillary Electrophoresis-Mass Spectrometry untargeted metabolomics with relative migration time and in-source fragmentation.
    Journal of chromatography. A, 2021, Jan-04, Volume: 1635

    Topics: Animals; Carnitine; Electrophoresis, Capillary; Humans; Metabolomics; Rabbits; Rats; Tandem Mass Spectrometry; Time Factors

2021
Carnitine Serum Levels in Frail Older Subjects.
    Nutrients, 2020, Dec-19, Volume: 12, Issue:12

    Topics: Aged; Aged, 80 and over; Carnitine; Cross-Sectional Studies; Female; Frail Elderly; Frailty; Geriatric Assessment; Humans; Male; Nutritional Status

2020
[Newborn Screening Program in the Community of Madrid: evaluation of positive cases.]
    Revista espanola de salud publica, 2020, Dec-16, Volume: 94

    Topics: Acyl-CoA Dehydrogenase; Amino Acid Metabolism, Inborn Errors; Carnitine; Cities; Female; Humans; Infant, Newborn; Lipid Metabolism, Inborn Errors; Male; Neonatal Screening; Predictive Value of Tests; Prevalence; Spain; Tandem Mass Spectrometry

2020
The Acylcarnitine Profile in Dried Blood Spots is Affected by Hematocrit: A Study of Newborn Screening Samples in Very-Low-Birth-Weight Infants.
    American journal of perinatology, 2022, Volume: 39, Issue:11

    Topics: Carnitine; Hematocrit; Humans; Infant; Infant, Newborn; Infant, Very Low Birth Weight; Neonatal Screening

2022
An Examination of Serum Acylcarnitine and Amino Acid Profiles at Different Time Point of Ketogenic Diet Therapy and Their Association of Ketogenic Diet Effectiveness.
    Nutrients, 2020, Dec-23, Volume: 13, Issue:1

    Topics: 3-Hydroxybutyric Acid; Adolescent; Amino Acids; Carnitine; Case-Control Studies; Child; Child, Preschool; Diet, Ketogenic; Drug Resistant Epilepsy; Female; Humans; Infant; Male; Prospective Studies; Tandem Mass Spectrometry; Treatment Outcome; Young Adult

2020
Walnut Consumption, Plasma Metabolomics, and Risk of Type 2 Diabetes and Cardiovascular Disease.
    The Journal of nutrition, 2021, 02-01, Volume: 151, Issue:2

    Topics: Amino Acids; Biomarkers; Cardiovascular Diseases; Carnitine; Diabetes Mellitus, Type 2; Humans; Juglans; Lipids; Metabolomics; Nuts; Purines; Risk Factors

2021
Hyperammonemia, Lactic Acidosis, and Arrhythmia in a Newborn.
    Clinical chemistry, 2021, 01-08, Volume: 67, Issue:1

    Topics: Acidosis, Lactic; Arrhythmias, Cardiac; Carnitine; Female; Humans; Hyperammonemia; Infant, Newborn; Lipid Metabolism, Inborn Errors; Membrane Transport Proteins; Mutation

2021
Exercise modifies glutamate and other metabolic biomarkers in cerebrospinal fluid from Gulf War Illness and Myalgic encephalomyelitis / Chronic Fatigue Syndrome.
    PloS one, 2021, Volume: 16, Issue:1

    Topics: Adult; Biomarkers; Carnitine; Case-Control Studies; Exercise Test; Fatigue Syndrome, Chronic; Female; Glutamic Acid; Humans; Male; Metabolomics; Middle Aged; Persian Gulf Syndrome; Phospholipases

2021
Untargeted Metabolomics Reveals Elevated L-Carnitine Metabolism in Pig and Rat Colon Tissue Following Red Versus White Meat Intake.
    Molecular nutrition & food research, 2021, Volume: 65, Issue:7

    Topics: Animals; Carnitine; Chickens; Colon; Diet, Western; HT29 Cells; Humans; Lipid Metabolism; Male; Metabolomics; Rats, Sprague-Dawley; Red Meat; Swine

2021
Gene spectrum and clinical traits of 10 patients with primary carnitine deficiency.
    Molecular genetics & genomic medicine, 2021, Volume: 9, Issue:2

    Topics: Adult; Cardiomyopathies; Carnitine; Female; Gene Frequency; Humans; Hyperammonemia; Infant; Male; Muscular Diseases; Mutation; Phenotype; Solute Carrier Family 22 Member 5

2021
Partial Biotinidase Deficiency Revealed Imbalances in Acylcarnitines Profile at Tandem Mass Spectrometry Newborn Screening.
    International journal of environmental research and public health, 2021, 02-09, Volume: 18, Issue:4

    Topics: Biotinidase; Biotinidase Deficiency; Carnitine; Female; Humans; Infant; Infant, Newborn; Neonatal Screening; Tandem Mass Spectrometry

2021
Circulating metabolites and lipids are associated with glycaemic measures in South Asians.
    Diabetic medicine : a journal of the British Diabetic Association, 2021, Volume: 38, Issue:2

    Topics: Adult; Aged; Aged, 80 and over; Asia, Western; Betaine; Blood Glucose; Carnitine; Chromatography, Liquid; Cohort Studies; Diabetes Mellitus; Diglycerides; Female; Glycated Hemoglobin; Humans; Linear Models; Lipidomics; Logistic Models; Male; Mass Spectrometry; Metabolomics; Middle Aged; Proline; Sphingomyelins; Triglycerides; United States

2021
Sex Differences in Insulin Sensitivity are Related to Muscle Tissue Acylcarnitine But Not Subcellular Lipid Distribution.
    Obesity (Silver Spring, Md.), 2021, Volume: 29, Issue:3

    Topics: Adult; Carnitine; Citric Acid Cycle; Cohort Studies; Female; Glucose Clamp Technique; Glucose Tolerance Test; Humans; Insulin; Insulin Resistance; Lipid Metabolism; Male; Mitochondria, Muscle; Muscle, Skeletal; Obesity; Oxidation-Reduction; Sex Characteristics; Sphingolipids; Subcellular Fractions

2021
Novel Strategy for Mining and Identification of Acylcarnitines Using Data-Independent-Acquisition-Based Retention Time Prediction Modeling and Pseudo-Characteristic Fragmentation Ion Matching.
    Journal of proteome research, 2021, 03-05, Volume: 20, Issue:3

    Topics: Carnitine; Chromatography, Liquid; Linear Models; Mass Spectrometry

2021
Rhodiola crenulata extract decreases fatty acid oxidation and autophagy to ameliorate pulmonary arterial hypertension by targeting inhibiton of acylcarnitine in rats.
    Chinese journal of natural medicines, 2021, Volume: 19, Issue:2

    Topics: Animals; Autophagy; Carnitine; Fatty Acids; Lipid Metabolism; Plant Extracts; Pulmonary Arterial Hypertension; Rats; Rhodiola

2021
Alterations in acylcarnitines, amines, and lipids inform about the mechanism of action of citalopram/escitalopram in major depression.
    Translational psychiatry, 2021, 03-02, Volume: 11, Issue:1

    Topics: Amines; Antidepressive Agents; Carnitine; Citalopram; Depression; Depressive Disorder, Major; Humans; Lipids; Selective Serotonin Reuptake Inhibitors

2021
Serum carnitine concentration and the acyl to free carnitine ratio in nondialysis chronic kidney disease and hemodialysis patients.
    Therapeutic apheresis and dialysis : official peer-reviewed journal of the International Society for Apheresis, the Japanese Society for Apheresis, the Japanese Society for Dialysis Therapy, 2022, Volume: 26, Issue:1

    Topics: Aged; Carnitine; Female; Humans; Male; Middle Aged; Renal Dialysis; Renal Insufficiency, Chronic

2022
Plasma Targeted Metabolomics Analysis for Amino Acids and Acylcarnitines in Patients with Prediabetes, Type 2 Diabetes Mellitus, and Diabetic Vascular Complications.
    Diabetes & metabolism journal, 2021, Volume: 45, Issue:2

    Topics: Amino Acids; Carnitine; Diabetes Mellitus, Type 2; Diabetic Angiopathies; Humans; Metabolomics; Prediabetic State

2021
Cell metabolomics analyses revealed a role of altered fatty acid oxidation in neurotoxicity pattern difference between nab-paclitaxel and solvent-based paclitaxel.
    PloS one, 2021, Volume: 16, Issue:3

    Topics: Acyl-CoA Dehydrogenase; Albumin-Bound Paclitaxel; Albumins; Carnitine; Cell Differentiation; Cell Line, Tumor; Cell Survival; Fatty Acids; Humans; Metabolomics; Neurotoxins; Oxidation-Reduction; Paclitaxel; Solvents

2021
Changes in the metabolic profile of human male postmortem frontal cortex and cerebrospinal fluid samples associated with heavy alcohol use.
    Addiction biology, 2021, Volume: 26, Issue:6

    Topics: Adult; Aged; Alcoholism; Autopsy; Body Mass Index; Carnitine; Cerebrospinal Fluid; Chromatography, Liquid; Frontal Lobe; Humans; Male; Mass Spectrometry; Middle Aged; Neurotransmitter Agents; Patient Acuity

2021
Perilipin 2 downregulation in β cells impairs insulin secretion under nutritional stress and damages mitochondria.
    JCI insight, 2021, 05-10, Volume: 6, Issue:9

    Topics: Animals; Carnitine; Diet, High-Fat; Down-Regulation; Glucose; Humans; In Vitro Techniques; Insulin Secretion; Insulin-Secreting Cells; Islets of Langerhans; Lipid Droplets; Mice; Mice, Knockout; Mitochondria; Oleic Acid; Oxidative Phosphorylation; Oxidative Stress; Oxygen Consumption; Perilipin-2; Rats; Stress, Physiological

2021
Newborn screening and molecular features of patients with multiple acyl-CoA dehydrogenase deficiency in Quanzhou, China.
    Journal of pediatric endocrinology & metabolism : JPEM, 2021, May-26, Volume: 34, Issue:5

    Topics: Acyl-CoA Dehydrogenase; Asian People; Carnitine; China; Female; Follow-Up Studies; Genetic Testing; Genotype; Humans; Infant, Newborn; Male; Multiple Acyl Coenzyme A Dehydrogenase Deficiency; Mutation; Neonatal Screening; Prognosis

2021
SLC22A14 is a mitochondrial riboflavin transporter required for sperm oxidative phosphorylation and male fertility.
    Cell reports, 2021, 04-20, Volume: 35, Issue:3

    Topics: Animals; Carnitine; Citric Acid Cycle; Diet; Fatty Acids; Female; Fertility; Fertilization in Vitro; Gene Expression; Humans; Infertility, Male; Male; Metabolome; Mice; Mice, Inbred C57BL; Mice, Knockout; Mitochondria; Mitochondrial Membranes; Models, Molecular; Organic Cation Transport Proteins; Oxidative Phosphorylation; Riboflavin; Sperm Motility; Spermatozoa

2021
Post-Mortem Metabolomics: A Novel Approach in Clinical Biomarker Discovery and a Potential Tool in Death Investigations.
    Chemical research in toxicology, 2021, 06-21, Volume: 34, Issue:6

    Topics: Amino Acids; Biomarkers; Carnitine; Chromatography, High Pressure Liquid; Discriminant Analysis; Humans; Lipids; Metabolomics; Niacinamide; Nucleotides; Principal Component Analysis; Steroids

2021
Association of plasma acylcarnitines with insulin sensitivity, insulin secretion, and prediabetes in a biracial cohort.
    Experimental biology and medicine (Maywood, N.J.), 2021, Volume: 246, Issue:15

    Topics: Adult; Blood Glucose; Carnitine; Cross-Sectional Studies; Glucose Intolerance; Humans; Insulin Resistance; Insulin Secretion; Insulins; Male; Middle Aged; Prediabetic State

2021
An Unusual Peak in a Common Clinical Presentation.
    Clinical chemistry, 2021, 04-29, Volume: 67, Issue:5

    Topics: Carnitine; Diagnosis, Differential; Female; Glutamate Formimidoyltransferase; Humans; Infant; Leukomalacia, Periventricular; Metabolism, Inborn Errors

2021
Increasing fatty acid oxidation elicits a sex-dependent response in failing mouse hearts.
    Journal of molecular and cellular cardiology, 2021, Volume: 158

    Topics: Acetyl-CoA Carboxylase; Animals; Carnitine; Disease Models, Animal; Energy Metabolism; Fatty Acids; Female; Gene Deletion; Heart Failure; Male; Mice; Mice, Inbred C57BL; Mice, Transgenic; Myocardium; Oxidation-Reduction; PPAR alpha; Sex Factors; Signal Transduction; Tamoxifen

2021
Metabolomics Profiling of Patients With A-β+ Ketosis-Prone Diabetes During Diabetic Ketoacidosis.
    Diabetes, 2021, Volume: 70, Issue:8

    Topics: Adult; Amino Acids, Branched-Chain; Autoantibodies; Carnitine; Diabetes Mellitus, Type 1; Diabetes Mellitus, Type 2; Diabetic Ketoacidosis; Female; Humans; Male; Metabolome; Metabolomics; Middle Aged

2021
    American journal of physiology. Endocrinology and metabolism, 2021, 07-01, Volume: 321, Issue:1

    Topics: Adrenergic beta-2 Receptor Agonists; Animals; Carnitine; Fatty Liver; Formoterol Fumarate; Gene Expression; Hepatic Stellate Cells; Lipid Metabolism; Lipogenesis; Liver; Male; Mice; Mice, Inbred C57BL; Non-alcoholic Fatty Liver Disease; Phosphatidate Phosphatase; Receptors, Adrenergic, beta-2; Triglycerides

2021
Long-chain acylcarnitine 18:1 acutely increases human atrial myocardial contractility and arrhythmia susceptibility.
    American journal of physiology. Heart and circulatory physiology, 2021, 07-01, Volume: 321, Issue:1

    Topics: Aged; Aged, 80 and over; Calcium Signaling; Carnitine; Female; Heart Atria; Humans; Male; Middle Aged; Myocardial Contraction; Myocytes, Cardiac

2021
Exacerbation of cardiovascular ageing by diabetes mellitus and its associations with acyl-carnitines.
    Aging, 2021, 06-04, Volume: 13, Issue:11

    Topics: Aged; Aging; Cardiovascular System; Carnitine; Diabetes Mellitus; Female; Humans; Male; Middle Aged

2021
Quantitation and evaluation of perinatal medium-chain and long-chain acylcarnitine blood concentrations in 12,000 full-term breastfed newborns.
    Journal of pediatric endocrinology & metabolism : JPEM, 2021, Aug-26, Volume: 34, Issue:8

    Topics: Biomarkers; Birth Weight; Breast Feeding; Carnitine; Female; Follow-Up Studies; Humans; Infant, Newborn; Lipid Metabolism, Inborn Errors; Male; Neonatal Screening; Prognosis

2021
Long-Chain Acylcarnitines Decrease the Phosphorylation of the Insulin Receptor at Tyr1151 Through a PTP1B-Dependent Mechanism.
    International journal of molecular sciences, 2021, Jun-16, Volume: 22, Issue:12

    Topics: Animals; Carnitine; CHO Cells; Cricetulus; Cyclic AMP-Dependent Protein Kinases; Insulin; Insulin Resistance; Models, Biological; Phosphorylation; Protein Phosphatase 2; Protein Tyrosine Phosphatase, Non-Receptor Type 1; Proto-Oncogene Proteins c-akt; Receptor, Insulin; Tyrosine

2021
Longitudinal associations of physical activity with plasma metabolites among colorectal cancer survivors up to 2 years after treatment.
    Scientific reports, 2021, 07-02, Volume: 11, Issue:1

    Topics: Aged; Arginine; Cancer Survivors; Carnitine; Citrulline; Colorectal Neoplasms; Exercise; Female; Histidine; Humans; Longitudinal Studies; Lysophosphatidylcholines; Male; Metabolome; Middle Aged; Quality of Life; Self Report; Sphingomyelins; Tandem Mass Spectrometry

2021
The metabolic landscape of decidua in recurrent pregnancy loss using a global metabolomics approach.
    Placenta, 2021, 09-01, Volume: 112

    Topics: Abortion, Habitual; Adult; Carnitine; Case-Control Studies; Decidua; Female; Glycerophospholipids; Group II Phospholipases A2; Humans; Lipidomics; Metabolome; Pregnancy; Sphingolipids; Transferases (Other Substituted Phosphate Groups)

2021
Increased plasma and milk short-chain acylcarnitine concentrations reflect systemic LPS response in mid-lactation dairy cows.
    American journal of physiology. Regulatory, integrative and comparative physiology, 2021, 09-01, Volume: 321, Issue:3

    Topics: Animals; Carnitine; Cattle; Diet; Dietary Supplements; Energy Metabolism; Fatty Acids; Lactation; Lipopolysaccharides; Metabolomics; Milk

2021
Circulating long chain acylcarnitines and outcomes in diabetic heart failure: an HF-ACTION clinical trial substudy.
    Cardiovascular diabetology, 2021, 08-03, Volume: 20, Issue:1

    Topics: Aged; Biomarkers; Carnitine; Clinical Trials as Topic; Diabetic Cardiomyopathies; Exercise Tolerance; Female; Health Status; Heart Failure; Hospitalization; Humans; Male; Metabolome; Metabolomics; Middle Aged; Prognosis; Risk Assessment; Risk Factors; Tandem Mass Spectrometry; Time Factors

2021
A longitudinal study of plasma acylcarnitines throughout pregnancy and associations with risk of gestational diabetes mellitus.
    Clinical nutrition (Edinburgh, Scotland), 2021, Volume: 40, Issue:8

    Topics: Adult; Biomarkers; Carnitine; Case-Control Studies; Diabetes, Gestational; Female; Gestational Age; Humans; Longitudinal Studies; Pregnancy; Pregnancy Trimesters; Prospective Studies; Risk Factors

2021
Acylcarnitine profile in Alaskan sled dogs during submaximal multiday exercise points out metabolic flexibility and liver role in energy metabolism.
    PloS one, 2021, Volume: 16, Issue:8

    Topics: Animals; Carnitine; Dogs; Energy Metabolism; Gluconeogenesis; Glycogen; Liver; Muscle, Skeletal; Physical Conditioning, Animal

2021
Association Between Graft Function and Urine CXCL10 and Acylcarnitines Levels in Kidney Transplant Recipients.
    Laboratory medicine, 2022, Jan-06, Volume: 53, Issue:1

    Topics: Carnitine; Chemokine CXCL10; Creatinine; Cystatin C; Graft Rejection; Humans; Kidney; Kidney Transplantation; Transplant Recipients

2022
The effect of combined oral contraceptives containing drospirenone and ethinylestradiol on serum levels of amino acids and acylcarnitines.
    Metabolomics : Official journal of the Metabolomic Society, 2021, 08-19, Volume: 17, Issue:9

    Topics: Amino Acids; Androstenes; Carnitine; Contraceptives, Oral, Combined; Ethinyl Estradiol; Female; Humans

2021
Nutrient-related metabolite profiles explain differences in body composition and size in Nile tilapia (Oreochromis niloticus) from different lakes.
    Scientific reports, 2021, 08-19, Volume: 11, Issue:1

    Topics: Amino Acids; Animals; Body Composition; Body Size; Carnitine; Chemical Phenomena; Cichlids; Fish Proteins; Food; Geography; Lakes; Lipids; Metabolomics

2021
Diminished retinal complex lipid synthesis and impaired fatty acid β-oxidation associated with human diabetic retinopathy.
    JCI insight, 2021, 10-08, Volume: 6, Issue:19

    Topics: Adult; Aged; American Indian or Alaska Native; Arizona; Black or African American; Carnitine; Case-Control Studies; Ceramides; Diabetes Mellitus, Type 2; Diabetic Retinopathy; Diglycerides; Disease Progression; Esters; Female; Hispanic or Latino; Humans; Lipidomics; Male; Mass Spectrometry; Middle Aged; Mitochondria; Phosphatidylcholines; Retina; Triglycerides; White People

2021
Long-Chain Acyl-Carnitines Interfere with Mitochondrial ATP Production Leading to Cardiac Dysfunction in Zebrafish.
    International journal of molecular sciences, 2021, Aug-06, Volume: 22, Issue:16

    Topics: Adenosine Triphosphate; Animals; Carnitine; Disease Models, Animal; Fatty Acids; Heart; Heart Diseases; Humans; Mitochondria, Heart; Myocytes, Cardiac; Oxidation-Reduction; Zebrafish

2021
Absolute Quantification of Acylcarnitines Using Integrated Tmt-PP Derivatization-Based LC-MS/MS and Quantitative Analysis of Multi-Components by a Single Marker Strategy.
    Analytical chemistry, 2021, 09-28, Volume: 93, Issue:38

    Topics: Animals; Carnitine; Chromatography, Liquid; Metabolomics; Mice; Tandem Mass Spectrometry

2021
Biological and Clinical Factors Contributing to the Metabolic Heterogeneity of Hospitalized Patients with and without COVID-19.
    Cells, 2021, 09-02, Volume: 10, Issue:9

    Topics: Acute Disease; Adult; Amino Acids; Body Mass Index; Carnitine; Cohort Studies; COVID-19; Fatty Acids; Female; Humans; Kynurenine; Machine Learning; Metabolomics; Middle Aged; Prognosis; Prospective Studies; SARS-CoV-2; Severity of Illness Index; Tryptophan

2021
Impact of Newborn Screening and Early Dietary Management on Clinical Outcome of Patients with Long Chain 3-Hydroxyacyl-CoA Dehydrogenase Deficiency and Medium Chain Acyl-CoA Dehydrogenase Deficiency-A Retrospective Nationwide Study.
    Nutrients, 2021, Aug-24, Volume: 13, Issue:9

    Topics: 3-Hydroxyacyl CoA Dehydrogenases; Acyl-CoA Dehydrogenase; Cardiomyopathies; Carnitine; Child; Child, Preschool; Czech Republic; Female; Humans; Incidence; Infant; Infant, Newborn; Lipid Metabolism, Inborn Errors; Male; Metabolism, Inborn Errors; Mitochondrial Myopathies; Mitochondrial Trifunctional Protein; Neonatal Screening; Nervous System Diseases; Outcome Assessment, Health Care; Retrospective Studies; Rhabdomyolysis; Severity of Illness Index

2021
Longitudinal Plasma Metabolomics Profile in Pregnancy-A Study in an Ethnically Diverse U.S. Pregnancy Cohort.
    Nutrients, 2021, Sep-01, Volume: 13, Issue:9

    Topics: Adolescent; Adult; Amino Acids; Body Mass Index; Carnitine; Fatty Acids; Female; Humans; Male; Metabolomics; Pregnancy; Pregnancy Trimesters; Prospective Studies; Racial Groups; United States; Young Adult

2021
Untargeted metabolomic analysis of urine samples for diagnosis of inherited metabolic disorders.
    Functional & integrative genomics, 2021, Volume: 21, Issue:5-6

    Topics: Amino Acids; Biomarkers; Carnitine; Child; Humans; Mass Spectrometry; Metabolic Diseases; Metabolomics; Nucleosides

2021
Spatial metabolomics identifies localized chemical changes in heart tissue during chronic cardiac Chagas Disease.
    PLoS neglected tropical diseases, 2021, Volume: 15, Issue:10

    Topics: Animals; Carnitine; Chagas Cardiomyopathy; Chronic Disease; Heart; Humans; Male; Metabolomics; Mice; Mice, Inbred C3H; Myocardium; Phosphorylcholine; Trypanosoma cruzi

2021
Impaired beta-oxidation increases vulnerability to influenza A infection.
    The Journal of biological chemistry, 2021, Volume: 297, Issue:5

    Topics: Amino Acids, Branched-Chain; Animals; Carnitine; Diabetes Mellitus, Experimental; Diabetes Mellitus, Type 2; Influenza A virus; Mice; Orthomyxoviridae Infections; Oxidation-Reduction

2021
Plasma acyl-carnitines, bilirubin, tyramine and tetrahydro-21-deoxycortisol in Parkinson's disease and essential tremor. A case control biomarker study.
    Parkinsonism & related disorders, 2021, Volume: 91

    Topics: Aged; Bilirubin; Biomarkers; Carnitine; Case-Control Studies; Cognition; Cortodoxone; Diagnosis, Differential; Essential Tremor; Female; Humans; Male; Middle Aged; Parkinson Disease; Tyramine

2021
Very long chain acylcarnitines and lysophosphatidylcholines in screening of peroxisomal disease in children by tandem mass spectrometry.
    Zhejiang da xue xue bao. Yi xue ban = Journal of Zhejiang University. Medical sciences, 2021, Aug-25, Volume: 50, Issue:4

    Topics: Carnitine; Child; Dried Blood Spot Testing; Humans; Lysophosphatidylcholines; Tandem Mass Spectrometry

2021
Medium & long-chain acylcarnitine's relation to lipid metabolism as potential predictors for diabetic cardiomyopathy: a metabolomic study.
    Lipids in health and disease, 2021, Nov-02, Volume: 20, Issue:1

    Topics: Adult; Aminoimidazole Carboxamide; Animals; Biomarkers; Carnitine; Cell Line; Diabetes Mellitus, Type 2; Diabetic Cardiomyopathies; Female; Humans; Lipid Metabolism; Male; Mass Spectrometry; Middle Aged; Myoblasts, Cardiac; Myristic Acids; Rats; Retrospective Studies; Ribonucleosides; Risk Factors

2021
Low cardiac content of long-chain acylcarnitines in TMLHE knockout mice prevents ischaemia-reperfusion-induced mitochondrial and cardiac damage.
    Free radical biology & medicine, 2021, Volume: 177

    Topics: Animals; Carnitine; Ischemia; Male; Mice; Mice, Knockout; Mitochondria, Heart; Reperfusion

2021
Revealing potential lipid biomarkers in clear cell renal cell carcinoma using targeted quantitative lipidomics.
    Lipids in health and disease, 2021, Nov-13, Volume: 20, Issue:1

    Topics: Adult; Biomarkers, Tumor; Carcinoma, Renal Cell; Carnitine; Cholesterol Esters; Chromatography, High Pressure Liquid; Diglycerides; Female; Humans; Kidney Neoplasms; Lipidomics; Lipids; Male; Middle Aged; Tandem Mass Spectrometry

2021
Intrauterine growth restriction elevates circulating acylcarnitines and suppresses fatty acid metabolism genes in the fetal sheep heart.
    The Journal of physiology, 2022, Volume: 600, Issue:3

    Topics: Animals; Carnitine; Fatty Acids; Female; Fetal Growth Retardation; Fetal Heart; Placenta; Pregnancy; Sheep

2022
Plasma Metabolomics of Intermediate and Neovascular Age-Related Macular Degeneration Patients.
    Cells, 2021, 11-12, Volume: 10, Issue:11

    Topics: Aged; Carnitine; Case-Control Studies; Discriminant Analysis; Female; Humans; Least-Squares Analysis; Linear Models; Lipids; Macular Degeneration; Male; Metabolic Networks and Pathways; Metabolome; Metabolomics; Middle Aged; Neovascularization, Pathologic

2021
Heterogenous Clinical Landscape in a Consanguineous Malonic Aciduria Family.
    International journal of molecular sciences, 2021, Nov-23, Volume: 22, Issue:23

    Topics: Carboxy-Lyases; Carnitine; Child, Preschool; Consanguinity; Homozygote; Humans; Male; Malonates; Malonyl Coenzyme A; Metabolism, Inborn Errors; Methylmalonic Acid; Pedigree; Point Mutation

2021
A clinically validated method to separate and quantify underivatized acylcarnitines and carnitine metabolic intermediates using mixed-mode chromatography with tandem mass spectrometry.
    Journal of chromatography. A, 2022, Jan-25, Volume: 1663

    Topics: Carnitine; Chromatography, Liquid; Humans; Isomerism; Tandem Mass Spectrometry

2022
Differential and Synergistic Effects of Low Birth Weight and Western Diet on Skeletal Muscle Vasculature, Mitochondrial Lipid Metabolism and Insulin Signaling in Male Guinea Pigs.
    Nutrients, 2021, Nov-29, Volume: 13, Issue:12

    Topics: Animal Nutritional Physiological Phenomena; Animals; Animals, Newborn; Birth Weight; Blood Glucose; Carnitine; Diet, Western; Disease Models, Animal; Female; Fetal Growth Retardation; Guinea Pigs; Insulin; Lipid Metabolism; Male; Mitochondria; Muscle, Skeletal; Placental Insufficiency; Pregnancy; Signal Transduction

2021
Gene and metabolite expression dependence on body mass index in human myocardium.
    Scientific reports, 2022, 01-26, Volume: 12, Issue:1

    Topics: Aged; Aged, 80 and over; Body Mass Index; Carnitine; Case-Control Studies; Cholesterol; Cohort Studies; Coronary Artery Bypass; Coronary Artery Disease; Female; Gene Expression Profiling; Humans; Immunity, Innate; Ketoglutaric Acids; Lipid Metabolism; Male; Metabolome; Middle Aged; Muscle Contraction; Myocardium; Obesity; Risk Factors; RNA, Messenger; Survival Analysis; Time Factors; Transcriptome

2022
A HILIC-MS/MS method development and validation for the quantitation of 13 acylcarnitines in human serum.
    Analytical and bioanalytical chemistry, 2022, Volume: 414, Issue:9

    Topics: Cardiovascular Diseases; Carnitine; Humans; Tandem Mass Spectrometry

2022
Correlation between Acylcarnitine and Peripheral Neuropathy in Type 2 Diabetes Mellitus.
    Journal of diabetes research, 2022, Volume: 2022

    Topics: Adult; Aged; Body Mass Index; Carnitine; China; Cross-Sectional Studies; Diabetes Mellitus, Type 2; Female; Humans; Male; Middle Aged; Peripheral Nervous System Diseases; Risk Factors

2022
Plasma acylcarnitine, risk for heart failure or atrial fibrillation, and effects of the Mediterranean diet or obesity.
    Revista espanola de cardiologia (English ed.), 2022, Volume: 75, Issue:8

    Topics: Atrial Fibrillation; Carnitine; Diet, Mediterranean; Heart Failure; Humans; Obesity

2022
Integrative metabolomic profiling reveals aberrations in myometrium associated with adenomyosis: a pilot study.
    Reproductive biology and endocrinology : RB&E, 2022, Mar-09, Volume: 20, Issue:1

    Topics: Adenomyosis; Adult; Amino Acids; Carbohydrate Metabolism; Carnitine; Chromatography, High Pressure Liquid; Female; Gas Chromatography-Mass Spectrometry; Humans; Lipid Metabolism; Mass Spectrometry; Metabolome; Metabolomics; Middle Aged; Myometrium; Nucleotides; Pilot Projects; Premenopause

2022
Altered Serum Acylcarnitines Profile after a Prolonged Stay in Intensive Care.
    Nutrients, 2022, Mar-07, Volume: 14, Issue:5

    Topics: Adolescent; Adult; Aged; Aged, 80 and over; Carnitine; Chromatography, Liquid; Critical Care; Female; Humans; Male; Middle Aged; Tandem Mass Spectrometry; Young Adult

2022
Correlation of Serum Acylcarnitines with Clinical Presentation and Severity of Coronary Artery Disease.
    Biomolecules, 2022, 02-23, Volume: 12, Issue:3

    Topics: Biomarkers; Carnitine; Coronary Artery Disease; Humans; Prospective Studies; ST Elevation Myocardial Infarction; Stroke Volume; Tandem Mass Spectrometry; Ventricular Function, Left

2022
Specific lysophosphatidylcholine and acylcarnitine related to sarcopenia and its components in older men.
    BMC geriatrics, 2022, 03-25, Volume: 22, Issue:1

    Topics: Aged; Amino Acids; Carnitine; Cross-Sectional Studies; Female; Hand Strength; Humans; Lysophosphatidylcholines; Male; Muscle, Skeletal; Sarcopenia

2022
Relationship Between Acylcarnitine and the Risk of Retinopathy in Type 2 Diabetes Mellitus.
    Frontiers in endocrinology, 2022, Volume: 13

    Topics: Biomarkers; Carnitine; Diabetes Mellitus, Type 2; Diabetic Retinopathy; Humans; ROC Curve

2022
NMR Characterization of Long-Chain Fatty Acylcarnitine Binding to the Mitochondrial Carnitine/Acylcarnitine Carrier.
    International journal of molecular sciences, 2022, Apr-21, Volume: 23, Issue:9

    Topics: Carnitine; Carnitine Acyltransferases; Magnetic Resonance Spectroscopy; Mitochondria

2022
Targeted metabolomics analysis of amino acids and acylcarnitines as risk markers for diabetes by LC-MS/MS technique.
    Scientific reports, 2022, 05-19, Volume: 12, Issue:1

    Topics: Amino Acids; Carnitine; Chromatography, Liquid; Diabetes Mellitus; Female; Humans; Male; Metabolomics; Tandem Mass Spectrometry

2022
Acylcarnitines and Genetic Variation in Fat Oxidation Genes in HIV-infected, Antiretroviral-treated Children With and Without Myopathy.
    The Pediatric infectious disease journal, 2022, 08-01, Volume: 41, Issue:8

    Topics: Anti-HIV Agents; Anti-Retroviral Agents; Carnitine; Child; Genetic Variation; HIV Infections; Humans; Muscular Diseases; Oxidation-Reduction; Reverse Transcriptase Inhibitors; Solute Carrier Family 22 Member 5

2022
Determinants of blood acylcarnitine concentrations in healthy individuals of the European Prospective Investigation into Cancer and Nutrition.
    Clinical nutrition (Edinburgh, Scotland), 2022, Volume: 41, Issue:8

    Topics: Carnitine; Diabetes Mellitus, Type 2; Fatty Acids; Humans; Neoplasms; Prospective Studies

2022
Inhibition of the carnitine acylcarnitine carrier by carbon monoxide reveals a novel mechanism of action with non-metal-containing proteins.
    Free radical biology & medicine, 2022, 08-01, Volume: 188

    Topics: Carbon Monoxide; Carnitine; Heme; Membrane Transport Proteins; Mitochondria; Organometallic Compounds

2022
Low carnitine palmitoyltransferase 1 activity is a risk factor for narcolepsy type 1 and other hypersomnia.
    Sleep, 2022, 10-10, Volume: 45, Issue:10

    Topics: Carnitine; Carnitine Acyltransferases; Carnitine O-Palmitoyltransferase; Disorders of Excessive Somnolence; Fatty Acids; Humans; Narcolepsy; Risk Factors

2022
Combining data acquisition modes in liquid-chromatography-tandem mass spectrometry for comprehensive determination of acylcarnitines in human serum.
    Metabolomics : Official journal of the Metabolomic Society, 2022, 07-21, Volume: 18, Issue:8

    Topics: Carnitine; Chromatography, Liquid; Humans; Metabolomics; Tandem Mass Spectrometry

2022
Circulating amino acids and acylcarnitines correlated with different CAC score ranges in diabetic postmenopausal women using LC-MS/MS based metabolomics approach.
    BMC endocrine disorders, 2022, Jul-21, Volume: 22, Issue:1

    Topics: Amino Acids; Cardiovascular Diseases; Carnitine; Chromatography, Liquid; Cross-Sectional Studies; Diabetes Mellitus; Female; Humans; Postmenopause; Tandem Mass Spectrometry

2022
Effects of propofol and dexmedetomidine on carnitine metabolism in normal human bronchial epithelial cells.
    Biotechnic & histochemistry : official publication of the Biological Stain Commission, 2023, Volume: 98, Issue:1

    Topics: Carnitine; Dexmedetomidine; Epithelial Cells; Humans; Propofol

2023
Effects of dietary branched-chain amino acid supplementation on serum and milk metabolome profiles in dairy cows during early lactation.
    Journal of dairy science, 2022, Volume: 105, Issue:10

    Topics: Amino Acids, Branched-Chain; Animals; Antioxidants; Carnitine; Cattle; Choline; Diet; Dietary Supplements; Fatty Acids; Female; Glucose; Glutathione; Humans; Insulins; Lactation; Lipids; Metabolome; Milk; Nitrogen; Phosphorylcholine; Urea

2022
Evaluating the association between amino acid and acylcarnitine profiles and different levels of coronary artery disease risk in postmenopausal women using targeted metabolomics technique.
    Menopause (New York, N.Y.), 2022, 09-01, Volume: 29, Issue:9

    Topics: Amino Acids; Biomarkers; Carnitine; Coronary Angiography; Coronary Artery Disease; Cross-Sectional Studies; Female; Humans; Phenylalanine; Postmenopause; Risk Factors; Vascular Calcification

2022
Tandem Mass Analysis of Amino Acids and Acylcarnitine Profiles in Neonates with Congenital Hypothyroidism.
    Endocrine, metabolic & immune disorders drug targets, 2023, Volume: 23, Issue:5

    Topics: Amino Acids; Carnitine; Case-Control Studies; Child; Congenital Hypothyroidism; Female; Humans; Infant, Newborn; Male

2023
Long time stability of 35 small endogenous biomolecules in dried urine spotted on various surfaces and environmental conditions.
    Forensic science international, 2022, Volume: 339

    Topics: Amino Acids; Body Fluids; Carnitine; Specimen Handling

2022
Melatonin Regulates the Daily Levels of Plasma Amino Acids, Acylcarnitines, Biogenic Amines, Sphingomyelins, and Hexoses in a Xenograft Model of Triple Negative Breast Cancer.
    International journal of molecular sciences, 2022, Aug-14, Volume: 23, Issue:16

    Topics: Amino Acids; Animals; Biogenic Amines; Carnitine; Heterografts; Hexoses; Humans; Melatonin; Mice; Sphingomyelins; Triple Negative Breast Neoplasms

2022
Identifying a metabolomics profile associated with masked hypertension in two independent cohorts: Data from the African-PREDICT and SABPA studies.
    Hypertension research : official journal of the Japanese Society of Hypertension, 2022, Volume: 45, Issue:11

    Topics: Amino Acids; Carnitine; Humans; Isoleucine; Leucine; Masked Hypertension; Metabolomics; Methionine; Phenylalanine; Valine

2022
Dynamics of acylcarnitines, hypoglycin A, méthylènecyclopropylglycine and their metabolites in a Kladruber stallion with atypical myopathy.
    The veterinary quarterly, 2022, Volume: 42, Issue:1

    Topics: Acyl-CoA Dehydrogenases; Animals; Carnitine; Creatine Kinase; Cyclopropanes; Fatty Acids; Glycine; Horse Diseases; Horses; Hypoglycins; Male; Muscular Diseases

2022
Proline/Glycine residues of the PG-levels guide conformational changes along the transport cycle in the mitochondrial carnitine/acylcarnitine carrier (SLC25A20).
    International journal of biological macromolecules, 2022, Nov-30, Volume: 221

    Topics: Alanine; Carnitine; Carnitine Acyltransferases; Glycine; Proline

2022
Tandem Mass Spectrometry for the Analysis of Plasma/Serum Acylcarnitines for the Diagnosis of Certain Organic Acidurias and Fatty Acid Oxidation Disorders.
    Methods in molecular biology (Clifton, N.J.), 2022, Volume: 2546

    Topics: Carnitine; Carnitine Acyltransferases; Fatty Acids; Humans; Lipid Metabolism, Inborn Errors; Tandem Mass Spectrometry

2022
Quantitation of Butyrylcarnitine, Isobutyrylcarnitine, and Glutarylcarnitine in Urine Using Ultra-Performance Liquid Chromatography-Tandem Mass Spectrometry (UPLC-MS/MS).
    Methods in molecular biology (Clifton, N.J.), 2022, Volume: 2546

    Topics: Carnitine; Chromatography, High Pressure Liquid; Chromatography, Liquid; Coenzyme A; Fatty Acids; Tandem Mass Spectrometry

2022
Quantification of Free and Total Carnitine in Serum Using Liquid Chromatography Tandem Mass Spectrometry.
    Methods in molecular biology (Clifton, N.J.), 2022, Volume: 2546

    Topics: Carnitine; Chromatography, Liquid; Fatty Acids; Hydrochloric Acid; Tandem Mass Spectrometry

2022
Targeted Metabolomics Revealed a Sex-Dependent Signature for Metabolic Syndrome in the Mexican Population.
    Nutrients, 2022, Sep-06, Volume: 14, Issue:18

    Topics: Carnitine; Citrulline; Cohort Studies; Female; Glycine; Humans; Male; Metabolic Syndrome; Metabolomics

2022
Cross-generational effects of maternal exposure to imazalil on anaerobic components and carnitine absorption associated with OCTN2 expression in mice.
    Chemosphere, 2022, Volume: 308, Issue:Pt 3

    Topics: Anaerobiosis; Animals; Caco-2 Cells; Carnitine; Cations; Female; Fungicides, Industrial; Humans; Imidazoles; Maternal Exposure; Mice; Mice, Inbred C57BL; Pregnancy; Solute Carrier Family 22 Member 5

2022
Early postnatal metabolic profile in neonates with critical CHDs.
    Cardiology in the young, 2023, Volume: 33, Issue:3

    Topics: Amino Acids; Carnitine; Case-Control Studies; Humans; Infant; Infant, Newborn; Metabolome

2023
Targeted metabolomic profiles of serum amino acids and acylcarnitines related to gastric cancer.
    PeerJ, 2022, Volume: 10

    Topics: Amino Acids; Arginine; Carnitine; Chromatography, Liquid; Gastritis; Gastritis, Atrophic; Humans; Stomach Neoplasms; Tandem Mass Spectrometry

2022
Evaluation of plasma carnitine status in patients diagnosed with juvenile idiopathic arthritis.
    Turkish journal of medical sciences, 2022, Volume: 52, Issue:3

    Topics: Amino Acids; Arthritis, Juvenile; Carnitine; Child; Fasting; Humans; Tandem Mass Spectrometry

2022
[Analysis of IVD gene variants in four children with isovalerate acidemia].
    Zhonghua yi xue yi chuan xue za zhi = Zhonghua yixue yichuanxue zazhi = Chinese journal of medical genetics, 2022, Dec-10, Volume: 39, Issue:12

    Topics: Acidosis; Carnitine; Child; Erythrocytes; High-Throughput Nucleotide Sequencing; Humans; Infant, Newborn

2022
Long-Chain Acylcarnitines Induce Senescence of Invariant Natural Killer T Cells in Hepatocellular Carcinoma.
    Cancer research, 2023, 02-15, Volume: 83, Issue:4

    Topics: Antigens, CD1d; Carcinoma, Hepatocellular; Carnitine; Cellular Senescence; Galactosylceramides; Humans; Liver Neoplasms; Natural Killer T-Cells

2023
Determination of Serum Differential Carnitine Ester Levels in HIV(+) Patients: A Cross-Sectional Study.
    Current HIV research, 2023, Volume: 21, Issue:1

    Topics: Carnitine; Chromatography, Liquid; Cross-Sectional Studies; Esters; HIV Infections; Humans; Tandem Mass Spectrometry

2023
Sphingolipids and acylcarnitines are altered in placentas from women with gestational diabetes mellitus.
    The British journal of nutrition, 2023, 09-28, Volume: 130, Issue:6

    Topics: Carnitine; Ceramides; Diabetes, Gestational; Female; Humans; Placenta; Pregnancy; Sphingolipids

2023
Metabolomics of a neonatal cohort from the Alliance for Maternal and Newborn Health Improvement biorepository: Effect of preanalytical variables on reference intervals.
    PloS one, 2023, Volume: 18, Issue:1

    Topics: Amines; Amino Acids; Carnitine; Female; Humans; Infant; Infant Health; Infant, Low Birth Weight; Infant, Newborn; Male

2023
Free amino acid and acylcarnitine values in Ursus americanus Pallas 1780 (black bear) from Northeastern Mexico.
    PloS one, 2023, Volume: 18, Issue:2

    Topics: Amino Acids; Animals; Carnitine; Mexico; Ursidae

2023
The association between acylcarnitine and amino acids profile and metabolic syndrome and its components in Iranian adults: Data from STEPs 2016.
    Frontiers in endocrinology, 2023, Volume: 14

    Topics: Adult; Carnitine; Chromatography, Liquid; Citrulline; Cross-Sectional Studies; Diabetes Mellitus, Type 2; Humans; Iran; Metabolic Syndrome; Tandem Mass Spectrometry; Trypsin

2023
Vitreous Fatty Amides and Acyl Carnitines Are Altered in Intermediate Age-Related Macular Degeneration.
    Investigative ophthalmology & visual science, 2023, 03-01, Volume: 64, Issue:3

    Topics: Amides; Carnitine; Humans; Macular Degeneration; Niacinamide; Succinates; Vitreous Body

2023
A case of Fukuyama-type congenital muscular dystrophy with acute carnitine deficiency triggered by fever, vomiting, and gastrointestinal bleeding.
    Nutrition (Burbank, Los Angeles County, Calif.), 2023, Volume: 110

    Topics: Amino Acids; Carnitine; Fatty Acids; Gastrointestinal Hemorrhage; Humans; Male; Muscular Atrophy; Muscular Dystrophies; Vomiting

2023
Heart-Type Fatty Acid Binding Protein Binds Long-Chain Acylcarnitines and Protects against Lipotoxicity.
    International journal of molecular sciences, 2023, Mar-14, Volume: 24, Issue:6

    Topics: Carnitine; Fatty Acid Binding Protein 3; Fatty Acid-Binding Proteins; Fatty Acids; Myocytes, Cardiac

2023
Modulation of sleep by trafficking of lipids through the
    eLife, 2023, 05-04, Volume: 12

    Topics: Animals; Blood-Brain Barrier; Carnitine; Drosophila; Drosophila melanogaster; Drosophila Proteins; Lipids; Membrane Transport Proteins; Sleep

2023
Predictive value of presepsin and acylcarnitines for severity and biliary drainage in acute cholangitis.
    World journal of gastroenterology, 2023, Apr-28, Volume: 29, Issue:16

    Topics: Acetylcarnitine; Biomarkers; C-Reactive Protein; Carnitine; Cholangitis; Drainage; Humans; Lipopolysaccharide Receptors; Peptide Fragments; Procalcitonin; Sepsis

2023
    Journal of ocular pharmacology and therapeutics : the official journal of the Association for Ocular Pharmacology and Therapeutics, 2023, Volume: 39, Issue:5

    Topics: Animals; Anterior Chamber; Carnitine; Swine

2023
High-Throughput Analysis of Underivatized Amino Acids and Acylcarnitines in Infant Serum: A Micromethod Based on Stable Isotope Dilution Targeted HILIC-ESI-MS/MS.
    Journal of agricultural and food chemistry, 2023, Jun-07, Volume: 71, Issue:22

    Topics: Amino Acids; Carnitine; Child; Child, Preschool; Humans; Infant; Reproducibility of Results; Tandem Mass Spectrometry

2023
Carnitine supplementation increases serum concentrations of free carnitine and total acylcarnitine in preterm neonates: A retrospective cohort study.
    JPEN. Journal of parenteral and enteral nutrition, 2023, Volume: 47, Issue:6

    Topics: Amino Acids; Carnitine; Dietary Supplements; Humans; Infant; Infant, Newborn; Lipids; Retrospective Studies

2023
Inhibition of the Mitochondrial Carnitine/Acylcarnitine Carrier by Itaconate through Irreversible Binding to Cysteine 136: Possible Pathophysiological Implications.
    Biomolecules, 2023, 06-15, Volume: 13, Issue:6

    Topics: Carnitine; Cysteine; HeLa Cells; Humans; Membrane Transport Modulators; Membrane Transport Proteins; Mitochondria

2023
Short and medium chain acylcarnitines as markers of outcome in diabetic and non-diabetic subjects with acute coronary syndromes.
    International journal of cardiology, 2023, 10-15, Volume: 389

    Topics: Acetylcarnitine; Acute Coronary Syndrome; Carnitine; Clinical Studies as Topic; Cohort Studies; Diabetes Mellitus; Humans; Multicenter Studies as Topic; Prospective Studies

2023
Metabolomic analysis of pig spleen reveals African swine fever virus infection increased acylcarnitine levels to facilitate viral replication.
    Journal of virology, 2023, 08-31, Volume: 97, Issue:8

    Topics: African Swine Fever; African Swine Fever Virus; Animals; Carnitine; Fatty Acids; Metabolomics; Spleen; Swine; Virus Replication

2023
Study of amphipathic metabolites in cardiac pathophysiology: Insights gained from long-chain acylcarnitines and calcium handling.
    Journal of cellular and molecular medicine, 2023, Volume: 27, Issue:22

    Topics: Calcium; Carnitine; Heart; Ryanodine Receptor Calcium Release Channel; Sarcoplasmic Reticulum

2023
Altered markers of mitochondrial function in adults with autism spectrum disorder.
    Autism research : official journal of the International Society for Autism Research, 2023, Volume: 16, Issue:11

    Topics: Adult; Autism Spectrum Disorder; Carnitine; Fatty Acids; Female; Humans; Lactic Acid; Male; Mitochondria; Pyruvic Acid

2023
[A case of very long chain acyl-CoA dehydrogenase deficiency diagnosed due to a trigger of hyperemesis gravidarum during pregnancy].
    Rinsho shinkeigaku = Clinical neurology, 2023, Oct-25, Volume: 63, Issue:10

    Topics: Acyl-CoA Dehydrogenase, Long-Chain; Adult; Carnitine; Fatty Acids; Female; Humans; Hyperemesis Gravidarum; Infant, Newborn; Lipid Metabolism, Inborn Errors; Pregnancy; Rhabdomyolysis

2023
Purification of S-adenosylmethionine: epsilon-N-L-lysine methyltransferase. The first enzyme in carnitine biosynthesis.
    The Journal of biological chemistry, 1977, Aug-25, Volume: 252, Issue:16

    Topics: Amino Acids; Carnitine; Lysine; Methyltransferases; Molecular Weight; Neurospora crassa; S-Adenosylmethionine; Spectrophotometry; Spectrophotometry, Ultraviolet

1977
Effects of long-term organ culture on neurotransmitter metabolism in the ganglia of Aplysia californica.
    Journal of neurobiology, 1974, Volume: 5, Issue:1

    Topics: Acetylcholinesterase; Acetyltransferases; Action Potentials; Amino Acids; Animals; Carboxy-Lyases; Carnitine; Catechol O-Methyltransferase; Choline; Electrophysiology; Evoked Potentials; Ganglia; Glucosephosphate Dehydrogenase; Nerve Tissue Proteins; Neurotransmitter Agents; Organ Culture Techniques; S-Adenosylmethionine; Synaptic Transmission; Time Factors

1974
Liquid cation exchange--a basis for sensitive radiometric assays for aromatic amino acid decarboxylases.
    Analytical biochemistry, 1972, Volume: 45, Issue:1

    Topics: Aminobutyrates; Animals; Carbon Isotopes; Carboxy-Lyases; Carnitine; Choline; Dihydroxyphenylalanine; Dopa Decarboxylase; Dopamine; Drosophila melanogaster; Glutamates; Histamine; Histidine; Hydrogen-Ion Concentration; Ion Exchange; Methods; Norepinephrine; Organophosphorus Compounds; Phenethylamines; S-Adenosylmethionine; Serotonin; Tritium; Tyramine; Tyrosine; Tyrosine Decarboxylase

1972