homocitrulline has been researched along with citrulline in 133 studies
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 36 (27.07) | 18.7374 |
| 1990's | 21 (15.79) | 18.2507 |
| 2000's | 23 (17.29) | 29.6817 |
| 2010's | 50 (37.59) | 24.3611 |
| 2020's | 3 (2.26) | 2.80 |
| Authors | Studies |
|---|---|
| Gatfield, PD; Haust, MD; Taller, E; Wolfe, DM | 1 |
| Dawson, LE; Kohrman, AF; Wilson, RW | 1 |
| Goto, I; Kobayashi, T; Shigeto, H; Yamada, T | 1 |
| Shimizu, H | 1 |
| Arbour, JF; Dubé, J; Flessas, J; Laberge, M; Lafleur, L; Lambert, MA; Lemay, JF; Mitchell, GA; Valle, D; Vanasse, M | 1 |
| Bordugo, A; Burlina, AB; Dionisi-Vici, C; Ferrari, V; Tuchman, M; Zacchello, F | 1 |
| Laframboise, R; Mandell, R; Pichette, J; Shih, VE | 1 |
| Brochu, P; Jasmin, G; Lambert, MA; Qureshi, IA; Seidman, EG; Smith, L | 1 |
| Azuma, T; Higa, S; Kishimoto, S; Sakoda, S; Suzuki, T; Tsujino, S | 1 |
| Imanari, T; Kobori, Y; Koshiishi, I | 1 |
| Imanari, T; Koshiishi, I | 1 |
| Knopman, DS; Shih, VE; Tuchman, M | 1 |
| Hayakawa, C; Kato, T; Mizutani, N; Sano, M | 1 |
| Kato, T; Mizutani, N; Sano, M | 2 |
| Botschner, J; Scriver, CR; Simell, O; Smith, DW | 1 |
| Bonnefont, JP; Chadefaux, B; Kamoun, P; Rabier, D; Saudubray, JM; Shih, VE | 1 |
| Alme, A; Broch, H; Gjessing, LR; Lie, SO; Lunde, HA; Undrum, T | 1 |
| Alvarez, L; Coude, FX; Fabregas, I; Fernandez Alvarez, E; Grimber, G; Pineda, M; Ribes, A; Rodes, M | 1 |
| Bachmann, C; Colombo, JP; Dionisi Vici, C; Gambarara, M; Sabetta, G | 1 |
| Oyanagi, K | 1 |
| Inoue, I; Kayanuma, K; Koike, R; Miyatake, T; Nakajima, M; Saheki, T; Sakoda, K; Takeshita, K; Uono, M; Yuasa, T | 1 |
| Hashiba, M; Noguchi, R; Shimoji, K; Tomita, Y | 1 |
| Akiyoshi, H; Ichihara, K; Inoue, I; Ishii, S; Mito, T; Nakajima, M; Saheki, T; Takakura, H; Takashima, S; Takeshita, K | 1 |
| Fujimori, K; Koike, R; Miyatake, T; Yuasa, T | 1 |
| Gatfield, DP; Gordon, BA; Haust, MD | 1 |
| Biscoping, J; Hempelmann, G; Michaelis, G | 1 |
| Dyken, PR; Hartlage, PL; Hommes, FA; Metoki, K; Roesel, RA | 1 |
| Clow, CL; Mackenzie, S; Scriver, CR; Simell, O | 1 |
| Gray, RG; Hill, SE; Pollitt, RJ | 1 |
| Perheentupa, J; Rajantie, J; Simell, O | 1 |
| Itakura, Y; Nakao, T; Oyanagi, K; Sakamoto, S; Sogawa, H; Tsuchiyama, A; Wagatsuma, K; Yachi, A | 1 |
| Hommes, FA; Metoki, K | 1 |
| Carter, AL; Eller, AG; Hommes, FA; Metoki, K; Rufo, S | 1 |
| Dyken, P; Hommes, FA; Kelloes, C; Metoki, K; Trefz, J | 1 |
| Carter, AL; Eller, AG; Evans, BA; Hommes, FA | 1 |
| Morimoto, K; Sato, M; Tanaka, A; Yamaha, T | 1 |
| Evered, DF; Vadgama, JV | 1 |
| Shih, VE | 1 |
| Herzfeld, A; Mandell, R; Shih, VE | 1 |
| Gordon, BA; Haust, MD | 1 |
| Harding, JJ; Rixon, KC | 1 |
| Kato, T; Sano, M | 1 |
| Griffith, OW; Narayanan, K | 1 |
| Gray, RG; Green, A; Hall, S; McKeown, C | 1 |
| Elberger, AJ; Handorf, CR; Kraus, AP; Kraus, LM; Pabst, MJ | 1 |
| Donati, MA; Filippi, L; Resti, M; Zammarchi, E | 1 |
| Lee, SC; Marekov, LN; Mei, G; Melino, G; Steinert, PM; Tarcsa, E | 1 |
| Bonocore, G; Buonocore, G; Ciani, F; Donati, MA; Pasquini, E; Shih, VE; Zammarchi, E | 1 |
| Nagao, M; Oyanagi, K | 1 |
| Boucher, JL; Mansuy, D; Moali, C; Sari, MA; Stuehr, DJ | 1 |
| Lammers, WL; Metwalli, AA; Van Boekel, MA | 1 |
| Celli, M; Crifò, C; D'Eufemia, P; Finocchiaro, R; Giardini, O; Salerno, C | 1 |
| Gallagher, AC; Pike, M; Standing, S | 1 |
| Gaber, L; Handorf, CR; Kraus, AP; Kraus, LM; Marti, HP | 1 |
| Ohura, T; Sakamoto, O | 1 |
| Bertini, E; Boldrini, R; Burlina, AB; Di Capua, M; Dionisi-Vici, C; Donati, A; Fariello, G; Meli, C; Rizzo, C; Salvi, S; Santorelli, FM | 1 |
| Bertini, E; Dionisi-Vici, C; Salvi, S; Santorelli, FM; Verardo, M | 1 |
| Dehne, MG; Fuchs, M; Hempelmann, G; Mühling, J; Sablotzki, A; Spatz, J; Weiss, S | 1 |
| Kakinuma, H | 1 |
| Kanazawa, N; Miyamoto, T; Tsujino, S | 1 |
| David, L; Dionisi-Vici, C; Dolce, V; Fiermonte, G; Palmieri, F; Santorelli, FM; Walker, JE | 1 |
| Andrade, D; Camacho, JA; Kong, J; Porter, J; Rioseco-Camacho, N | 1 |
| GERRITSEN, T; LIPTON, SH; STRONG, FM; WAISMAN, HA | 2 |
| GERRITSEN, T; VAUGHN, JG; WAISMAN, HA | 1 |
| RYAN, WL; WELLS, IC | 1 |
| Abu-Libdeh, B; Gutman, A; Kanazawa, N; Korman, SH; Tsujino, S | 1 |
| Andria, G; Carbone, MT; Correra, A; Della Casa, R; Dionisi-Vici, C; Fecarotta, S; Parenti, G; Riva, S; Santorelli, FM; Torre, G; Vajro, P; Zuppaldi, A | 1 |
| Al-Dirbashi, OY; Al-Hassnan, ZN; Rashed, MS | 1 |
| Abu-Amero, KK; Al-Dirbashi, OY; Al-Hassnan, ZN; Patay, Z; Rahbeeni, Z; Rashed, MS | 1 |
| Barnard, J; DiDonato, JA; Hazen, SL; Hörkkö, S; Kummu, O; Nicholls, SJ; Reynolds, WF; Rodriguez, ER; Topol, EJ; Wang, Z | 1 |
| Ischiropoulos, H; Rader, DJ | 1 |
| Chan, A; Collison, M; Greenberg, CR; Huynh, HQ; Lehotay, DC; Mhanni, AA; Seifert, B; Sokoro, A | 1 |
| Jelkmann, W | 1 |
| Debray, FG; Drouin, R; Dubé, J; Fenyves, D; Laframboise, R; Lambert, M; Lemieux, B; Maranda, B; Mitchell, GA; Soucy, JF | 1 |
| Aldamiz-Echevarría, L; Baumgartner, MR; Besley, G; Chien, YH; de Baulny, HO; Deodato, F; Dionisi-Vici, C; Fiermonte, G; Hernandez, JM; Loguercio, C; Martinez-Hernandez, E; Melone, MA; Nassogne, MC; Palmieri, F; Paradies, E; Parenti, G; Pierri, CL; Rutledge, SL; Santorelli, FM; Scarano, G; Schiff, M; Tessa, A; Vilaseca, MA; Walter, J | 1 |
| Amaral, AU; Dutra Filho, CS; Ferreira, Gda C; Knebel, LA; Schuck, PF; Tonin, AM; Viegas, CM; Wajner, M; Wannmacher, CM; Zanatta, A | 1 |
| Boenzi, S; Capozzi, P; Dionisi-Vici, C; Morini, C; Rizzo, C; Santorelli, FM | 1 |
| Amaral, AU; Dutra-Filho, CS; Fernandes, CG; Leipnitz, G; Seminotti, B; Viegas, CM; Wajner, M; Zanatta, A | 1 |
| Ghaffari, MA; Shanaki, M | 1 |
| Bokarewa, M; Brisslert, M; Dahlberg, LE; Hazen, SL; Hellvard, A; Mydel, P; Wang, Z | 1 |
| Koivula, MK; Risteli, J; Risteli, L; Turunen, S | 2 |
| Busanello, EN; de Moura, AP; Ferreira, Gda C; Grings, M; Ritter, L; Schuck, PF; Sitta, A; Tonin, AM; Vargas, CR; Viegas, CM; Wajner, M | 1 |
| Cerami, A; Huizinga, TW; Janssen, GM; Knevel, R; Levarht, NE; Shi, J; Suwannalai, P; Toes, RE; Trouw, LA; van der Helm-van Mil, AH; van der Linden, MP; van Veelen, PA | 1 |
| Gillery, P; Gorisse, L; Jaisson, S; Pietrement, C | 1 |
| Garnotel, R; Guilbert, M; Jeannesson, P; Millerot-Serrurot, E; Said, G; Terryn, C; Van Gulick, L | 1 |
| Busanello, EN; Grings, M; Knebel, LA; Leipnitz, G; Lobato, VA; Moura, AP; Pettenuzzo, LF; Ritter, L; Tonin, AM; Vargas, CR; Viegas, CM; Wajner, M; Zanatta, A | 1 |
| Busanello, EN; Fernandes, CG; Leipnitz, G; Moura, AP; Seminotti, B; Tonin, AM; Viegas, CM; Wajner, M; Zanatta, A | 1 |
| Cerami, A; Drijfhout, JW; Huizinga, TW; Janssen, GM; Shi, J; Toes, RE; Trouw, LA; van Veelen, PA; Willemze, A | 1 |
| Delporte, C; Desmet, JM; Dufour, D; Franck, T; Madhoun, P; Moguilevsky, N; Nève, J; Nortier, J; Noyon, C; Raes, M; Rousseau, A; Serteyn, D; Van Antwerpen, P; Vanhaeverbeek, M; Vanhamme, L; Zouaoui Boudjeltia, K | 1 |
| van Schaardenburg, D | 1 |
| Huizinga, TW; Toes, RE; Trouw, LA; Willemze, A | 1 |
| Huizinga, TW; Toes, RE; Trouw, LA | 1 |
| Azize, NA; Ch'ng, GS; Chen, BC; Habib, A; Hsu, HT; Md Yunus, Z; Ngu, LH; Ong, WP; Pitt, J; Wong, KJ | 1 |
| Fu, X; Hazen, SL; Kalantar-Zadeh, K; Koeth, RA; Tang, WH; Wang, Z | 1 |
| Borowski, AG; Hazen, SL; Klein, AL; Shrestha, K; Tang, WH; Troughton, RW; Wang, Z | 1 |
| Busanello, EN; Grings, M; Leipnitz, G; Moura, AP; Tonin, AM; Viegas, CM; Wajner, M; Zanatta, A | 1 |
| Alasaarela, E; Koivula, MK; Lehenkari, P; Melkko, J; Risteli, J; Turunen, S | 1 |
| Barra, L; Bell, DA; Cairns, E; Gladman, DD; Joseph, R; McCormick, JK; Pope, J; Racapé, M; Scinocca, M; Shaw, G | 1 |
| El-Gamal, D; Frank, S; Gauster, M; Hallström, S; Haybaeck, J; Heinemann, A; Holzer, M; Kozina, A; Marsche, G; Rao, SP; Schicho, R; Schuligoi, R; Wadsack, C | 1 |
| Bergum, B; Bielecka, E; Binder, V; Blom, AM; Brun, JG; Dahl-Knudsen, A; Enghild, JJ; Hellvard, A; Jonsson, R; Koro, C; Mydel, P; Potempa, J; Scavenius, C | 1 |
| Addad, F; Gillery, P; Hammami, M; Jaisson, S; Kerkeni, M; Santos-Weiss, IC | 1 |
| Arstila, L; Elfving, P; Häyrynen, J; Kaipiainen-Seppänen, O; Kärkkäinen, M; Kautiainen, H; Koivula, MK; Kononoff, A; Niinisalo, H; Risteli, J; Savolainen, E | 1 |
| Hannonen, P; Koivula, MK; Risteli, J; Risteli, L; Turunen, S | 1 |
| Bracke, KR; Brusselle, GG; Correia, RE; Fischer, R; Kessler, BM; Lugli, EB; Lundberg, K; Montgomery, AB; Venables, PJ | 1 |
| Desmons, A; Gillery, P; Jaisson, S; Pietrement, C; Rieu, P; Wynckel, A | 1 |
| Elferink, CJ; Joshi, AD; Lichti, CF; Mustafa, MG | 1 |
| El-Gabalawy, HS; Hitchon, CA; Huizinga, TJ; Koppejan, H; Lahey, LJ; Robinson, DB; Smolik, IA; Sokolove, J; Toes, RE; Trouw, LA | 1 |
| Amaral, AU; Quincozes-Santos, A; Rodrigues, MD; Souza, DG; Wajner, M; Zanatta, Â | 1 |
| Desmons, A; Fadel, F; Gillery, P; Jaisson, S; Kazes, I; Millart, H; Oudart, JB; Rieu, P; Santos-Weiss, IC; Touré, F | 1 |
| Alten, R; Bang, H; Cobra, JF; Englbrecht, M; Feuchtenberger, M; Figueiredo, CP; Finzel, S; Fleck, M; Haschka, J; Henes, J; Hueber, AJ; Kleinert, S; Kleyer, A; Krueger, K; Lorenz, HM; Manger, B; Manger, K; Nuesslein, H; Ochs, W; Rech, J; Reiser, M; Ronneberger, M; Schett, G; Schmitt-Haendle, M; Schuch, F; Tony, HP; Wendler, J | 1 |
| Aronica, MA; Buffa, J; Comhair, SA; DiDonato, JA; Dweik, RA; Erzurum, SC; Hazen, SL; Kavuru, M; Lee, JJ; Lee, NA; Thomassen, MJ; Wang, Z | 1 |
| Huhtakangas, J; Lehenkari, P; Melkko, J; Nousiainen, T; Risteli, J; Turunen, S; Valkealahti, M | 1 |
| Lu, L; Mao, JY; Shen, WF; Sun, JT; Wang, YP; Wu, LP; Wu, QH; Yang, K; Zhang, RY | 1 |
| Bang, H; Burmester, GR; Camici, G; Detert, J; Feist, E; Gay, S; Ghannam, K; Keller, S; Krämer, A; Ospelt, C | 1 |
| Becker, T; Hanff, E; Tsikas, D | 1 |
| Drijfhout, JW; Janssen, GM; Mahler, M; Seaman, A; Toes, REM; Trouw, LA; van Veelen, PA; Verheul, MK; Yee, A | 1 |
| Hamilton, BJ; Jones, JD; Rigby, WFC | 1 |
| Bergum, B; Binder, V; Chapple, ILC; Delaleu, N; Gillery, P; Hellvard, A; Jaisson, S; Mydel, P; Nyhaug, AK; Roberts, HM; Scavenius, C; Spriet, E | 1 |
| de Ru, AH; Dekkers, JS; Hegen, M; Huizinga, TWJ; Ioan-Facsinay, A; Janssen, GMC; Liu, B; Rapecki, S; Stoop, JN; Toes, REM; Trouw, LA; van Veelen, PA; Verheul, MK | 1 |
| Hamada, C; Hattori, S; Kusubata, M; Ogawa-Goto, K; Taga, Y; Tanaka, K | 1 |
| Coresh, J; Grams, ME; Inker, LA; Köttgen, A; Levey, AS; Perrone, RD; Rebholz, CM; Sarnak, MJ; Shafi, T; Tin, A | 1 |
| Fielding, RA; Lustgarten, MS | 1 |
| Barra, L; Bell, DA; Cairns, E; Lac, P; Racapé, M | 1 |
| Barra, L; Bell, DA; Cairns, E; Lac, P; Saunders, S; Tutunea-Fatan, E | 1 |
| Coremans, C; Delporte, C; Dieu, M; Ducobu, J; Dufour, D; Furtmüller, PG; Maki, RA; Moguilevsky, N; Nève, J; Noyon, C; Nuyens, V; Obinger, C; Raes, M; Reye, F; Reynolds, WF; Robaye, B; Rousseau, A; Soudi, M; Van Antwerpen, P; Vanhaeverbeek, M; Vanhamme, L; Zouaoui Boudjeltia, K | 1 |
| De Simone, E; Di Iorio, B; Di Micco, L; La Manna, G | 1 |
| Desmons, A; Doué, M; Gillery, P; Gorisse, L; Jaisson, S; Pietrement, C | 1 |
| Bang, H; Bonger, KM; Cavallari, M; Drijfhout, JW; Huizinga, T; Janssen, G; Kampstra, A; Kissel, T; Kwekkeboom, JC; Levarht, E; Reijm, S; Reth, M; Scherer, HU; Slot, LM; Stoeken-Rijsbergen, G; Toes, R; van Veelen, PA; Vergroesen, RD; Wortel, CM | 1 |
| Borbély, A; Schlosser, G; Steckel, A; Uray, K | 1 |
| Atabani, S; Boocock, D; Brentville, VA; Choudhury, RH; Cook, KW; Coveney, C; Daniels, I; Durrant, LG; Gijon, M; Metheringham, RL; Miles, AK; Shah, S; Symonds, P; Vaghela, P; Weston, D; Xue, W | 1 |
9 review(s) available for homocitrulline and citrulline
| Article | Year |
|---|---|
Hyperornithinemia, hyperammonemia, homocitrullinuria (HHH) syndrome: presentation as acute liver disease with coagulopathy.
Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Blood Coagulation Disorders; Child, Preschool; Citrulline; Female; Humans; Liver Diseases; Liver Function Tests; Ornithine; Syndrome | 1992 |
Regulation of ornithine metabolism.
Topics: Ammonia; Animals; Biological Transport; Citrulline; Cytosol; Fibroblasts; Humans; Mitochondria; Mitochondria, Liver; Ornithine; Ornithine Carbamoyltransferase; Urea | 1981 |
[Hyperornithinemia-hyperammonemia-homocitrullinuria (H.H.H.) syndrome].
Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Citrulline; Diagnosis, Differential; Humans; Infant, Newborn; Ornithine; Ornithine-Oxo-Acid Transaminase; Syndrome | 1998 |
[Hyperornithinemia-hyperammonemia-homocitrullinuria syndrome].
Topics: Citrulline; Humans; Hyperammonemia; Ornithine; Syndrome | 2001 |
[Structure-function relationships of mitochondrial transporters].
Topics: Animals; Citrulline; Humans; Hyperammonemia; Intracellular Membranes; Membrane Transport Proteins; Mitochondria; Mitochondrial ADP, ATP Translocases; Mutation; Ornithine; Phosphate Transport Proteins; Syndrome | 2002 |
[Mitochondrial ornithine transporter deficiency].
Topics: Amino Acid Transport Systems, Basic; Carrier Proteins; Citrulline; Diagnosis, Differential; Humans; Hyperammonemia; Membrane Transport Proteins; Mitochondria; Mitochondrial Membrane Transport Proteins; Mutation; Ornithine; Prognosis; Proteins; Syndrome | 2002 |
New biomarkers in rheumatoid arthritis.
Topics: Antirheumatic Agents; Arthritis, Rheumatoid; Autoantibodies; Biomarkers; Citrulline; Humans; Prognosis | 2012 |
Autoimmunity in rheumatoid arthritis: different antigens--common principles.
Topics: Antibody Formation; Arthritis, Rheumatoid; Autoantigens; Autoimmunity; Citrulline; Humans | 2013 |
[Protein carbamylation: what it is and why it concerns nephrologists].
Topics: Alzheimer Disease; Amino Acids; Amyloidosis; Anemia, Sickle Cell; Animals; Cardiovascular Diseases; Cataract; Chromatography, High Pressure Liquid; Citrulline; Clinical Trials as Topic; Cyanates; Follow-Up Studies; Humans; Kidney Failure, Chronic; Lipoproteins; Protein Carbamylation; Renal Dialysis; Tandem Mass Spectrometry; tau Proteins; Urea | 2018 |
124 other study(ies) available for homocitrulline and citrulline
| Article | Year |
|---|---|
Hyperornithinemia, hyperammonemia, and homocitrullinuria associated with decreased carbamyl phosphate synthetase I activity.
Topics: Adolescent; Adult; Amino Acid Metabolism, Inborn Errors; Amino Acids; Ammonia; Biopsy; Carbamoyl-Phosphate Synthase (Ammonia); Child; Child, Preschool; Citrulline; Dietary Proteins; Female; Humans; Leukocytes; Liver; Lysine; Male; Microscopy, Electron; Mitochondria, Liver; Ornithine; Phosphotransferases | 1975 |
A new metabolic product of L-homocitrulline.
Topics: Animals; Chromatography, Paper; Citrulline; Male; Rats | 1977 |
[A case of hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome with spastic paraparesis and severe distal muscle atrophy of lower limbs].
Topics: Adolescent; Amino Acid Metabolism, Inborn Errors; Ammonia; Citrulline; Electromyography; Humans; Leg; Male; Muscular Atrophy; Ornithine; Paraparesis, Tropical Spastic; Spinal Cord; Syndrome | 1992 |
[The dibasic amino acid metabolic disorders].
Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Arginine; Blood Transfusion; Citrulline; Humans; Hyperargininemia; Ornithine; Ornithine Carbamoyltransferase Deficiency Disease; Renal Dialysis | 1992 |
Hyperammonemia-hyperornithinemia-homocitrullinuria syndrome: neurologic, ophthalmologic, and neuropsychologic examination of six patients.
Topics: Adolescent; Adult; Amino Acid Metabolism, Inborn Errors; Ammonia; Child; Child, Preschool; Citrulline; Eye Diseases; Female; Humans; Male; Nervous System Diseases; Neuropsychological Tests; Ornithine; Psychomotor Performance | 1992 |
Allopurinol challenge test in children.
Topics: Adolescent; Aging; Allopurinol; Child; Child, Preschool; Citrulline; Female; Humans; Infant; Male; Metabolism, Inborn Errors; Ornithine Carbamoyltransferase Deficiency Disease; Orotic Acid; Urea; Uridine | 1992 |
Neonatal form of the hyperornithinaemia, hyperammonaemia, and homocitrullinuria (HHH) syndrome and prenatal diagnosis.
Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Cells, Cultured; Citrulline; Female; Gestational Age; Hepatitis B Surface Antigens; Humans; Hyaline Membrane Disease; Infant, Newborn; Male; Ornithine; Ornithine Carbamoyltransferase Deficiency Disease; Ornithine-Oxo-Acid Transaminase; Pregnancy; Pregnancy Outcome; Prenatal Diagnosis; Syndrome | 1992 |
Hyperornithinemia, hyperammonemia and homocitrullinuria--a case report and study of ornithine metabolism using in vivo deuterium labelling.
Topics: Adult; Ammonia; Arginine; Chromatography, Liquid; Citrulline; Deuterium; Humans; Male; Mass Spectrometry; Ornithine | 1991 |
Determination of citrulline and homocitrulline by high-performance liquid chromatography with post-column derivatization.
Topics: Adult; Chromatography, High Pressure Liquid; Citrulline; Female; Humans; Male; Reference Values | 1990 |
State analysis of endogenous cyanate ion in human plasma.
Topics: Adult; Aged; Blood Proteins; Carbamates; Citrulline; Cyanates; Erythrocytes; Female; Humans; Lysine; Male; Middle Aged; Renal Dialysis; Sulfhydryl Compounds; Urea | 1990 |
Episodic hyperammonemia in adult siblings with hyperornithinemia, hyperammonemia, and homocitrullinuria syndrome.
Topics: Adult; Ammonia; Citrulline; Female; Fibroblasts; Glutamine; Humans; Leucine; Male; Metabolism, Inborn Errors; Ornithine; Phenylbutyrates; Recurrence; Syndrome; Urea | 1990 |
Homocitrullinuria and homoargininuria in hyperargininaemia.
Topics: Amino Acid Metabolism, Inborn Errors; Arginine; Child; Child, Preschool; Citrulline; Homoarginine; Humans; Lysine; Male | 1988 |
Homocitrullinuria and homoargininuria in lysinuric protein intolerance.
Topics: Adolescent; Amino Acid Metabolism, Inborn Errors; Arginine; Child; Citrulline; Female; Homoarginine; Humans; Kidney; Lysine; Male; Ornithine | 1989 |
Comparison of ornithine metabolism in hyperornithinaemia-hyperammonaemia-homocitrullinuria syndrome, lysinuric protein intolerance and gyrate atrophy fibroblasts.
Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Citrulline; Fibroblasts; Heterozygote; Homozygote; Humans; Lysine; Ornithine; Ornithine-Oxo-Acid Transaminase; Oxidation-Reduction; Proteins; Syndrome | 1989 |
Potential for the prenatal diagnosis of hyperornithinemia, hyperammonemia, and homocitrullinuria syndrome.
Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Chorionic Villi Sampling; Citrulline; Female; Humans; Ornithine; Pregnancy; Pregnancy Trimester, First; Prenatal Diagnosis | 1989 |
A new patient with hyperornithinaemia, hyperammonaemia and homocitrullinuria treated early with low protein diet.
Topics: Amino Acid Metabolism, Inborn Errors; Amino Acids; Ammonia; Citrulline; Dietary Proteins; Glutamine; Humans; Infant; Male; Ornithine; Pyrimidines | 1986 |
A new family affected by the syndrome of hyperornithinaemia, hyperammonaemia and homocitrullinuria.
Topics: Adolescent; Adult; Amino Acid Metabolism, Inborn Errors; Amino Acids; Ammonia; Citrulline; Female; Humans; Male; Ornithine; Syndrome | 1987 |
Inhibitory effect of intravenous lysine infusion on urea cycle metabolism.
Topics: Adolescent; Amino Acids; Ammonia; Child; Citric Acid Cycle; Citrulline; Depression, Chemical; Female; Humans; Infusions, Intravenous; Lysine; Male; Orotic Acid; Putrescine; Urea | 1987 |
Hyperornithinemia-hyperammonemia-homocitrullinuria syndrome: low creatine excretion and effect of citrulline, arginine, or ornithine supplement.
Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Arginine; Child, Preschool; Citrulline; Creatine; Female; Humans; Lysine; Male; Ornithine; Orotic Acid; Syndrome | 1987 |
[Hyperornithinemia, hyperammonemia and homocitrullinuria due to a defect of mitochondrial membrane transport].
Topics: Amino Acids; Ammonia; Biological Transport; Cells, Cultured; Citrulline; Fibroblasts; Humans; Mitochondria, Liver; Ornithine; Skin | 1988 |
Biochemical analysis of decreased ornithine transport activity in the liver mitochondria from patients with hyperornithinemia, hyperammonemia and homocitrullinuria.
Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Biological Transport; Citrulline; Humans; Kinetics; Mitochondria, Liver; Ornithine | 1988 |
[Anesthesia for a patient with the hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome].
Topics: Adult; Ammonia; Anesthesia; Citrulline; Humans; Male; Ornithine; Syndrome | 1988 |
Clinical, biochemical and ultrastructural study on the pathogenesis of hyperornithinemia-hyperammonemia-homocitrullinuria syndrome.
Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Arginine; Brain; Child; Citrulline; Endoplasmic Reticulum; Humans; Liver Glycogen; Male; Mitochondria, Liver; Ornithine; Tomography, X-Ray Computed | 1988 |
[Hyperornithinemia, hyperammonemia and homocitrullinuria syndrome in a family].
Topics: Adolescent; Adult; Amino Acid Metabolism, Inborn Errors; Ammonia; Citrulline; Electroencephalography; Humans; Male; Mitochondria; Ornithine; Syndrome | 1987 |
The hyperornithinemia, hyperammonemia, homocitrullinuria syndrome: an ornithine transport defect remediable with ornithine supplements.
Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Child; Citrulline; Dietary Proteins; Fibroblasts; Humans; In Vitro Techniques; Male; Mitochondria; Ornithine; Oxidation-Reduction; Syndrome | 1987 |
[H-H-H syndrome: a rare disease also relevant in anesthesiology].
Topics: Adolescent; Ammonia; Anesthesia, General; Citrulline; Femoral Fractures; Fracture Fixation, Internal; Humans; Male; Ornithine; Postoperative Complications; Syndrome; Wound Healing | 1986 |
Studies on a case of HHH-syndrome (hyperammonemia, hyperornithinemia, homocitrullinuria).
Topics: Amino Acid Metabolism, Inborn Errors; Amino Acids; Ammonia; Biological Transport; Child, Preschool; Citrulline; Fibroblasts; Follow-Up Studies; Humans; Kinetics; Male; Ornithine; Psychomotor Disorders; Seizures; Syndrome | 1986 |
Ornithine loading did not prevent induced hyperammonemia in a patient with hyperornithinemia-hyperammonemia-homocitrullinuria syndrome.
Topics: Alanine; Amino Acid Metabolism, Inborn Errors; Amino Acids; Ammonia; Child; Citrulline; Cytoplasm; Humans; Male; Ornithine; Orotic Acid; Syndrome | 1985 |
Reduced ornithine catabolism in cultured fibroblasts and phytohaemagglutinin-stimulated lymphocytes from a patient with hyperornithinaemia, hyperammonaemia and homocitrullinuria.
Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Aspartate Ammonia-Lyase; Cells, Cultured; Citrulline; Fibroblasts; Glutamates; Glutamic Acid; Humans; Lymphocyte Activation; Lymphocytes; Ornithine; Phytohemagglutinins; Proline | 1982 |
Oral administration of epsilon N-acetyllysine and homocitrulline in lysinuric protein intolerance.
Topics: Administration, Oral; Adolescent; Adult; Amino Acid Metabolism, Inborn Errors; Child; Child, Preschool; Citrulline; Female; Humans; Lysine; Male | 1983 |
The mechanism of hyperammonaemia and hyperornithinaemia in the syndrome of hyperornithinaemia, hyperammonaemia with homocitrullinuria.
Topics: Ammonia; Citrulline; Fibroblasts; Humans; Liver; Male; Middle Aged; Mitochondria, Liver; Ornithine; Syndrome | 1983 |
The pH of mitochondria of fibroblasts from a hyperornithinaemia, hyperammonaemia, homocitrullinuria-syndrome patient.
Topics: Acid-Base Equilibrium; Ammonia; Citrulline; Cytosol; Fibroblasts; Humans; Hydrogen-Ion Concentration; Mitochondria; Ornithine; Syndrome | 1984 |
Further evidence for a separate enzymic entity for the synthesis of homocitrulline, distinct from the regular ornithine transcarbamylase.
Topics: Animals; Citrulline; Female; Hydrogen-Ion Concentration; Kinetics; Lysine; Male; Mice; Mice, Mutant Strains; Mitochondria, Liver; Ornithine; Ornithine Carbamoyltransferase; Rats | 1984 |
Ultrastructural changes in fibroblast mitochondria of a patient with HHH-syndrome.
Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Citrulline; Fibroblasts; Genes, Recessive; Humans; Intracellular Membranes; Microscopy, Electron; Mitochondria; Ornithine; Skin | 1984 |
Reconstitution of ornithine transport in liposomes with Lubrol extracts of mitochondria.
Topics: Amino Acid Transport Systems, Basic; Ammonia; Animals; Biological Transport, Active; Carrier Proteins; Cattle; Citrulline; Kinetics; Liposomes; Membrane Transport Proteins; Mitochondria, Liver; Ornithine; Polyethylene Glycols; Solubility; Syndrome | 1984 |
Modification of poly-L-lysine by treatment with 1,1-ethylene-bis(1-nitrosourea) in vitro.
Topics: Chemical Phenomena; Chemistry; Citrulline; Lysine; Nitrosourea Compounds; Polylysine | 1981 |
Absorption of homocitrulline from the gastrointestinal tract.
Topics: Absorption; Adult; Amino Acids; Amobarbital; Animals; Citrulline; Ethacrynic Acid; Female; Humans; In Vitro Techniques; Intestinal Absorption; Intestine, Small; Male; Mouth Mucosa; Rats; Sodium | 1983 |
Defective ornithine metabolism in cultured skin fibroblasts from patients with the syndrome of hyperornithinemia, hyperammonemia and homocitrullinuria.
Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Cells, Cultured; Citrulline; Fibroblasts; Humans; Models, Chemical; Ornithine; Proteins; Skin | 1982 |
Ultrastructural changes in the mitochondria in disorders in ornithine metabolism.
Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Citrulline; Humans; Mitochondria, Liver; Ornithine; Ornithine Carbamoyltransferase Deficiency Disease | 1980 |
Carbamylation of lens proteins: a possible factor in cataractogenesis in some tropical countries.
Topics: Animals; Cataract; Cattle; Citrulline; Crystallins; Cyanates; Diarrhea; In Vitro Techniques; Molecular Weight; Pakistan; Uremia | 1980 |
Effect of ammonium chloride on homocitrulline and homoarginine synthesis from lysine.
Topics: Adolescent; Ammonium Chloride; Child; Citrulline; Homoarginine; Humans; Lysine | 1993 |
Synthesis of L-thiocitrulline, L-homothiocitrulline, and S-methyl-L-thiocitrulline: a new class of potent nitric oxide synthase inhibitors.
Topics: Amino Acid Oxidoreductases; Aminocaproates; Aminocaproic Acid; Animals; Binding Sites; Brain; Citrulline; Heme; Iron; Muscle, Smooth; Nitric Oxide Synthase; Rats; Thiourea | 1994 |
Prenatal exclusion of the HHH syndrome.
Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Amniotic Fluid; Cells, Cultured; Citrulline; Female; Humans; Ornithine; Pregnancy; Prenatal Diagnosis; Syndrome | 1995 |
Urea-derived cyanate forms epsilon-amino-carbamoyl-lysine (homocitrulline) in leukocyte proteins in patients with end-stage renal disease on peritoneal dialysis.
Topics: Adult; Aged; Blood Proteins; Blood Urea Nitrogen; Citrulline; Cyanates; Female; Fluorescein-5-isothiocyanate; Fluorescent Antibody Technique; Fluorescent Dyes; Humans; Kidney Failure, Chronic; Leukocytes; Male; Middle Aged; Neutrophils; Peritoneal Dialysis; Superoxides; Urea | 1994 |
Cryptogenic hepatitis masking the diagnosis of ornithine transcarbamylase deficiency.
Topics: Absorption; Ammonia; Arginine; Child, Preschool; Citrulline; Diagnosis, Differential; Dietary Proteins; Female; Glutamine; Hepatitis; Humans; Infant; Lysine; Ornithine; Ornithine Carbamoyltransferase Deficiency Disease; Orotic Acid; Urea | 1996 |
Protein unfolding by peptidylarginine deiminase. Substrate specificity and structural relationships of the natural substrates trichohyalin and filaggrin.
Topics: Amino Acid Sequence; Animals; Arginine; Circular Dichroism; Citrulline; Filaggrin Proteins; Humans; Hydrolases; Intermediate Filament Proteins; Mice; Molecular Sequence Data; Peptides; Protein Denaturation; Protein Precursors; Protein Structure, Secondary; Protein-Arginine Deiminase Type 4; Protein-Arginine Deiminases; Skin; Spectrometry, Fluorescence; Substrate Specificity | 1996 |
Neonatal onset of hyperornithinemia-hyperammonemia-homocitrullinuria syndrome with favorable outcome.
Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Benzoates; Benzoic Acid; Carnitine; Citrulline; Diet, Protein-Restricted; Humans; Infant, Newborn; Male; Ornithine | 1997 |
Substrate specificity of NO synthases: detailed comparison of L-arginine, homo-L-arginine, their N omega-hydroxy derivatives, and N omega-hydroxynor-L-arginine.
Topics: Animals; Arginine; Catalysis; Cattle; Citrulline; Homoarginine; Kinetics; NADP; Nitric Oxide Synthase; Nitrites; Oxidation-Reduction; Oxygen; Substrate Specificity | 1998 |
Formation of homocitrulline during heating of milk.
Topics: Amino Acids; Animals; Chromatography, Ion Exchange; Citrulline; Hot Temperature; Hydrolysis; Kinetics; Milk; Milk Proteins; Urea | 1998 |
Determination of urinary orotic acid and uracil by capillary zone electrophoresis.
Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Borates; Buffers; Citrulline; Dietary Proteins; Electrophoresis, Capillary; Humans; Hydrogen-Ion Concentration; Infant; Male; Ornithine; Orotic Acid; Syndrome; Uracil | 1999 |
HHH syndrome associated with callosal agenesis and disordered neuronal migration.
Topics: Abnormalities, Multiple; Agenesis of Corpus Callosum; Bronchopulmonary Sequestration; Citrulline; Consanguinity; Female; Humans; Hyperammonemia; Infant; Metabolism, Inborn Errors; Ornithine; Phenotype; Syndrome | 2001 |
Carbamoylation of glomerular and tubular proteins in patients with kidney failure: a potential mechanism of ongoing renal damage.
Topics: Amino Acids; Animals; Carbamates; Citrulline; Cyanates; Disease Progression; Fluorescent Antibody Technique; Humans; Immunohistochemistry; Kidney Failure, Chronic; Kidney Transplantation; Matrix Metalloproteinase 2; Microscopy, Fluorescence; Proteins; Rats | 2001 |
Clinical and molecular findings in hyperornithinemia-hyperammonemia-homocitrullinuria syndrome.
Topics: Adolescent; Adult; Brain Diseases, Metabolic, Inborn; Child; Citrulline; Female; Humans; Hyperammonemia; Male; Mitochondria, Muscle; Mutation; Ornithine; Paraparesis, Spastic; Retrospective Studies | 2001 |
Seven novel mutations in the ORNT1 gene (SLC25A15) in patients with hyperornithinemia, hyperammonemia, and homocitrullinuria syndrome.
Topics: Age of Onset; Alleles; Amino Acid Transport Systems, Basic; Base Sequence; Citrulline; DNA Mutational Analysis; Exons; Female; Fibroblasts; Humans; Hyperammonemia; Infant, Newborn; Italy; Male; Metabolism, Inborn Errors; Mitochondrial Membrane Transport Proteins; Mutation; Ornithine; Pedigree; Polymorphism, Restriction Fragment Length; Proteins; Reverse Transcriptase Polymerase Chain Reaction; Syndrome | 2001 |
Conscientious metabolic monitoring on a patient with hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome undergoing anaesthesia.
Topics: Adult; Amino Acids; Ammonia; Anesthesia; Blood Coagulation Tests; Citrulline; Clinical Chemistry Tests; Female; Humans; Monitoring, Physiologic; Ornithine; Syndrome | 2001 |
The mitochondrial ornithine transporter. Bacterial expression, reconstitution, functional characterization, and tissue distribution of two human isoforms.
Topics: Amino Acid Transport Systems, Basic; Arginine; Biological Transport; Carrier Proteins; Citrulline; DNA, Complementary; Electrophoresis, Polyacrylamide Gel; Humans; Hyperammonemia; Kinetics; Liposomes; Liver; Lysine; Malates; Membrane Transport Proteins; Mitochondria; Models, Biological; Mutation; Ornithine; Phosphates; Protein Folding; Protein Isoforms; Recombinant Proteins; Reverse Transcriptase Polymerase Chain Reaction; Substrate Specificity; Time Factors; Tissue Distribution; Transcription, Genetic | 2003 |
Cloning and characterization of human ORNT2: a second mitochondrial ornithine transporter that can rescue a defective ORNT1 in patients with the hyperornithinemia-hyperammonemia-homocitrullinuria syndrome, a urea cycle disorder.
Topics: Amino Acid Sequence; Amino Acid Transport Systems, Basic; Cell Line; Citrulline; Cloning, Molecular; Fibroblasts; Gene Frequency; Humans; Hyperammonemia; Membrane Transport Proteins; Mitochondrial Membrane Transport Proteins; Molecular Sequence Data; Ornithine; Polymorphism, Genetic; Proteins; Syndrome | 2003 |
On the isolation and identification of homocitrulline from urine.
Topics: Amino Acids; Body Fluids; Citrulline; Humans | 1961 |
Natural occurrence of homocitrulline. I. Excretion in the urine.
Topics: Amino Acids; Biological Transport; Citrulline; Humans | 1962 |
The origin of homocitrulline in the urine of infants.
Topics: Amino Acids; Animals; Citrulline; Humans; Infant; Milk | 1963 |
HOMOCITRULLINE AND HOMOARGININE SYNTHESIS FROM LYSINE.
Topics: Adult; Amino Acids; Body Fluids; Carbon Isotopes; Carbon Radioisotopes; Chromatography; Citrulline; Homoarginine; Humans; Kidney; Liver; Lysine; Male; Proteins; Rats; Research; Urine | 1964 |
Hyperornithinemia, hyperammonemia, and homocitrullinuria syndrome with evidence of mitochondrial dysfunction due to a novel SLC25A15 (ORNT1) gene mutation in a Palestinian family.
Topics: Adolescent; Alanine; Amino Acid Metabolism, Inborn Errors; Amino Acid Transport Systems, Basic; Arabs; Child, Preschool; Citrulline; DNA Mutational Analysis; Exons; Family Health; Gas Chromatography-Mass Spectrometry; Glycine; Humans; Hyperammonemia; Lactic Acid; Male; Mitochondrial Diseases; Mitochondrial Membrane Transport Proteins; Mutation; Ornithine; Polymerase Chain Reaction; Proteins | 2004 |
HHH syndrome (hyperornithinaemia, hyperammonaemia, homocitrullinuria), with fulminant hepatitis-like presentation.
Topics: Alanine Transaminase; Amino Acid Transport Systems, Basic; Arginine; Aspartate Aminotransferases; Child, Preschool; Citrulline; Diet, Protein-Restricted; Hepatitis; Humans; Hyperammonemia; Liver; Male; Metabolism, Inborn Errors; Mitochondrial Membrane Transport Proteins; Ornithine; Proteins; Syndrome | 2006 |
Determination of homocitrulline in urine of patients with HHH syndrome by liquid chromatography tandem mass spectrometry.
Topics: Ammonia; Chromatography, Liquid; Citrulline; Creatinine; Humans; Mass Spectrometry; Ornithine; Syndrome | 2006 |
Hyperornithinemia-hyperammonemia-homocitrullinuria syndrome with stroke-like imaging presentation: clinical, biochemical and molecular analysis.
Topics: Adolescent; Amino Acid Metabolism, Inborn Errors; Amino Acid Transport Systems, Basic; Brain; Brain Diseases, Metabolic, Inborn; Child; Child, Preschool; Citrulline; DNA Mutational Analysis; Female; Food, Formulated; Genetic Markers; Genetic Predisposition to Disease; Genetic Testing; Genotype; Humans; Hyperammonemia; Magnetic Resonance Imaging; Male; Mitochondrial Membrane Transport Proteins; Mutation; Ornithine; Pedigree; Saudi Arabia; Sodium Benzoate; Stroke | 2008 |
Protein carbamylation links inflammation, smoking, uremia and atherogenesis.
Topics: Animals; Atherosclerosis; Blood Proteins; Carbamates; Cholesterol; Citrulline; Clinical Trials as Topic; Coronary Artery Disease; Cyanates; Dose-Response Relationship, Drug; Female; Foam Cells; Humans; Inflammation; Jurkat Cells; Lipoproteins, LDL; Macrophages; Male; Mice; Mice, Inbred C57BL; Mice, Knockout; Mice, Transgenic; Models, Biological; Oxidation-Reduction; Peroxidase; Predictive Value of Tests; Protein Processing, Post-Translational; Receptors, LDL; Scavenger Receptors, Class A; Smoking; Uremia | 2007 |
'Multipurpose oxidase' in atherogenesis.
Topics: Animals; Apoptosis; Atherosclerosis; Carbamates; Cardiovascular Diseases; Cell Proliferation; Citrulline; Endothelial Cells; Humans; Inflammation; Lipoproteins, HDL; Lipoproteins, LDL; Lysine; Macrophages; Mice; Models, Biological; Muscle, Smooth; Oxidation-Reduction; Peroxidase; Scavenger Receptors, Class A; Smoking; Substrate Specificity; Thiocyanates | 2007 |
Hyperornithinemia-hyperammonemia-homocitrullinuria syndrome (HHH) presenting with acute fulminant hepatic failure.
Topics: Amino Acid Transport Systems, Basic; Citrulline; Diet, Protein-Restricted; Female; Humans; Hyperammonemia; Infant; Liver Failure, Acute; Liver Transplantation; Metabolism, Inborn Errors; Mitochondrial Membrane Transport Proteins; Ornithine; Point Mutation; Syndrome | 2008 |
'O', erythropoietin carbamoylation versus carbamylation.
Topics: Citrulline; Epoetin Alfa; Erythropoietin; Kidney; Kidney Tubules; Recombinant Proteins; Terminology as Topic; Ureteral Obstruction | 2008 |
Phenotypic variability among patients with hyperornithinaemia-hyperammonaemia-homocitrullinuria syndrome homozygous for the delF188 mutation in SLC25A15.
Topics: Adolescent; Amino Acid Metabolism, Inborn Errors; Amino Acid Transport Systems, Basic; Child; Child, Preschool; Citrulline; Founder Effect; Homozygote; Humans; Hyperammonemia; Infant; Mutation; Ornithine; Phenotype; Syndrome | 2008 |
Identification of novel mutations in the SLC25A15 gene in hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome: a clinical, molecular, and functional study.
Topics: Adult; Amino Acid Transport Systems, Basic; Biological Transport; Child; Child, Preschool; Citrulline; Escherichia coli; Female; Humans; Hyperammonemia; Infant; Infant, Newborn; Male; Middle Aged; Mitochondrial Membrane Transport Proteins; Mutant Proteins; Mutation; Ornithine; Protein Structure, Secondary; Syndrome | 2009 |
Experimental evidence that ornithine and homocitrulline disrupt energy metabolism in brain of young rats.
Topics: Analysis of Variance; Animals; Cerebral Cortex; Citric Acid Cycle; Citrulline; Creatine Kinase; Cytosol; Electron Transport Chain Complex Proteins; In Vitro Techniques; Mitochondria; Ornithine; Orotic Acid; Oxygen Consumption; Rats; Sodium-Potassium-Exchanging ATPase; Spectrophotometry; Synaptic Membranes | 2009 |
Retinal degeneration.
Topics: Adult; Amino Acid Transport Systems, Basic; Cataract; Citrulline; Consanguinity; Creatine; Diet, Protein-Restricted; Female; Humans; Hyperammonemia; Metabolism, Inborn Errors; Mitochondrial Membrane Transport Proteins; Ornithine; Retinal Degeneration; Syndrome; Vision Disorders | 2009 |
Evidence that the major metabolites accumulating in hyperornithinemia-hyperammonemia-homocitrullinuria syndrome induce oxidative stress in brain of young rats.
Topics: Animals; Antioxidants; Brain; Citrulline; Glutathione; Humans; Hyperammonemia; Lipid Peroxidation; Male; Nitric Oxide; Ornithine; Oxidation-Reduction; Oxidative Stress; Rats; Rats, Wistar; Syndrome; Thiobarbituric Acid Reactive Substances | 2009 |
In vitro inhibition of low density lipoprotein carbamylation by vitamins, as an ameliorating atherosclerotic risk in uremic patients.
Topics: Adult; alpha-Tocopherol; Ascorbic Acid; Atherosclerosis; Carotenoids; Citrulline; Cyanates; Electrophoresis, Agar Gel; Humans; Lipoproteins, LDL; Lycopene; Male; Protein Processing, Post-Translational; Uremia; Vitamins; Young Adult | 2010 |
Carbamylation-dependent activation of T cells: a novel mechanism in the pathogenesis of autoimmune arthritis.
Topics: Adoptive Transfer; Adult; Aged; Aged, 80 and over; Animals; Arthritis, Experimental; Arthritis, Rheumatoid; Cell Separation; Citrulline; Cytokines; Enzyme-Linked Immunosorbent Assay; Female; Flow Cytometry; Humans; Lymphocyte Activation; Male; Mice; Mice, Inbred BALB C; Mice, Inbred C57BL; Middle Aged; Protein Processing, Post-Translational; T-Lymphocytes | 2010 |
Anticitrulline antibodies can be caused by homocitrulline-containing proteins in rabbits.
Topics: Animals; Antibodies; Antibody Formation; Blotting, Western; Chromatography, High Pressure Liquid; Citrulline; Male; Rabbits | 2010 |
Dual mechanism of brain damage induced in vivo by the major metabolites accumulating in hyperornithinemia-hyperammonemia-homocitrullinuria syndrome.
Topics: Animals; Cerebral Cortex; Citrulline; Hyperammonemia; Injections, Intraventricular; Lipid Peroxidation; Ornithine; Oxidative Stress; Rats; Rats, Wistar; Urea Cycle Disorders, Inborn | 2011 |
Autoantibodies recognizing carbamylated proteins are present in sera of patients with rheumatoid arthritis and predict joint damage.
Topics: Arthritis, Rheumatoid; Autoantibodies; Autoantigens; Biomarkers; Blood Proteins; Case-Control Studies; Citrulline; Humans; Immunoglobulin A; Immunoglobulin G; Joints | 2011 |
Quantification of plasma homocitrulline using hydrophilic interaction liquid chromatography (HILIC) coupled to tandem mass spectrometry.
Topics: Animals; Chromatography, Liquid; Citrulline; Female; Hydrophobic and Hydrophilic Interactions; Linear Models; Mice; Mice, Inbred C57BL; Sensitivity and Specificity; Tandem Mass Spectrometry; Uremia | 2012 |
Impact of carbamylation and glycation of collagen type I on migration of HT1080 human fibrosarcoma cells.
Topics: Actins; Amino Acid Sequence; Cell Adhesion; Cell Line, Tumor; Cell Movement; Cell Proliferation; Cell Shape; Citrulline; Collagen Type I; Cytoskeleton; Enzyme Activation; Fibrosarcoma; Focal Adhesion Protein-Tyrosine Kinases; Glycosylation; Humans; Microscopy, Video; Molecular Sequence Data; Protein Processing, Post-Translational; Single-Cell Analysis; Time-Lapse Imaging; Vinculin | 2012 |
Chronic postnatal ornithine administration to rats provokes learning deficit in the open field task.
Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Animals; Animals, Newborn; Avoidance Learning; Behavior, Animal; Citrulline; Cognition; Developmental Disabilities; Disease Models, Animal; Half-Life; Learning Disabilities; Maze Learning; Memory; Memory, Long-Term; Ornithine; Postural Balance; Rats; Rats, Wistar; Thiobarbituric Acid Reactive Substances | 2012 |
Impairment of brain redox homeostasis caused by the major metabolites accumulating in hyperornithinemia-hyperammonemia-homocitrullinuria syndrome in vivo.
Topics: Amino Acid Metabolism, Inborn Errors; Ammonia; Animals; Antioxidants; Brain; Catalase; Citrulline; Glutathione Peroxidase; Homeostasis; Injections, Intraperitoneal; Injections, Intraventricular; Male; Nitric Oxide; Ornithine; Oxidation-Reduction; Oxidative Stress; Protein Carbonylation; Rats; Rats, Wistar; Sulfhydryl Compounds; Thiobarbituric Acid Reactive Substances; Urease | 2012 |
Recognition of citrullinated and carbamylated proteins by human antibodies: specificity, cross-reactivity and the 'AMC-Senshu' method.
Topics: Antibody Specificity; Autoantibodies; Blotting, Western; Citrulline; Cross Reactions; Humans; Proteins; Sensitivity and Specificity | 2013 |
Simultaneous measurement of protein-bound 3-chlorotyrosine and homocitrulline by LC-MS/MS after hydrolysis assisted by microwave: application to the study of myeloperoxidase activity during hemodialysis.
Topics: Aged; Aged, 80 and over; Animals; Blood Chemical Analysis; Blood Proteins; Chromatography, Liquid; Citrulline; Female; Humans; Hydrolysis; Lysine; Male; Microwaves; Middle Aged; Peroxidase; Renal Dialysis; Tandem Mass Spectrometry; Time Factors; Tyrosine | 2012 |
Gauging rheumatoid arthritis.
Topics: 14-3-3 Proteins; Arthritis, Rheumatoid; Autoantibodies; Biomarkers; Citrulline; Humans; Rheumatoid Factor | 2012 |
Hyperexcretion of homocitrulline in a Malaysian patient with lysinuric protein intolerance.
Topics: Amino Acid Metabolism, Inborn Errors; Amino Acid Transport System y+L; Biomarkers; Child, Preschool; Citrulline; Fusion Regulatory Protein 1, Light Chains; Genetic Markers; Genetic Testing; Humans; Malaysia; Male; Point Mutation | 2013 |
Protein carbamylation predicts mortality in ESRD.
Topics: Adult; Aged; Atherosclerosis; Citrulline; Cohort Studies; Female; Humans; Kidney Failure, Chronic; Male; Middle Aged; Prognosis; Protein Binding; Protein Processing, Post-Translational; Proteins; Renal Dialysis | 2013 |
Protein carbamylation in chronic systolic heart failure: relationship with renal impairment and adverse long-term outcomes.
Topics: Adult; Aged; Blood Proteins; Chronic Disease; Citrulline; Cohort Studies; Female; Heart Failure, Systolic; Humans; Kidney Diseases; Male; Middle Aged; Prospective Studies; Protein Binding; Time Factors; Treatment Outcome | 2013 |
Disturbance of redox homeostasis by ornithine and homocitrulline in rat cerebellum: a possible mechanism of cerebellar dysfunction in HHH syndrome.
Topics: Aconitate Hydratase; Animals; Cerebellum; Citrulline; Creatine Kinase; Electron Transport; Glutathione; Homeostasis; Hydrogen Peroxide; Hyperammonemia; Ketoglutarate Dehydrogenase Complex; Nerve Tissue Proteins; Nitrates; Nitrites; Ornithine; Oxidation-Reduction; Rats; Rats, Wistar; Sodium-Potassium-Exchanging ATPase; Thiobarbituric Acid Reactive Substances; Urea Cycle Disorders, Inborn | 2013 |
Different amounts of protein-bound citrulline and homocitrulline in foot joint tissues of a patient with anti-citrullinated protein antibody positive erosive rheumatoid arthritis.
Topics: Arthritis, Rheumatoid; Autoantibodies; Chromatography, High Pressure Liquid; Citrulline; Female; Foot Joints; Humans; Middle Aged; Protein Binding; Radiography | 2013 |
Antihomocitrullinated fibrinogen antibodies are specific to rheumatoid arthritis and frequently bind citrullinated proteins/peptides.
Topics: Adult; Aged; Aged, 80 and over; Arthritis, Rheumatoid; Autoantibodies; Citrulline; Epitopes; Female; Fibrinogen; Humans; Male; Middle Aged; Young Adult | 2014 |
The urea decomposition product cyanate promotes endothelial dysfunction.
Topics: Administration, Inhalation; Animals; Aorta; Cells, Cultured; Citrulline; Cyanates; Dose-Response Relationship, Drug; Endothelial Cells; Endothelium, Vascular; Humans; Male; Mice, Inbred C57BL; Nitric Oxide Synthase Type III; Phenotype; Plasminogen Activator Inhibitor 1; Protein Processing, Post-Translational; RNA, Messenger; Thromboplastin; Time Factors; Vasodilation; Vasodilator Agents | 2014 |
Carbamylation of immunoglobulin abrogates activation of the classical complement pathway.
Topics: Adhesins, Bacterial; Antibodies, Monoclonal, Murine-Derived; Antibody-Dependent Cell Cytotoxicity; Arthritis, Rheumatoid; Citrulline; Complement Activation; Complement C1q; Complement Pathway, Classical; Cyanates; Cysteine Endopeptidases; Gingipain Cysteine Endopeptidases; Humans; Immunoglobulin G; Immunologic Factors; Inflammation; Lysine; Mass Spectrometry; Protein Binding; Protein Processing, Post-Translational; Rituximab | 2014 |
Increased serum homocitrulline concentrations are associated with the severity of coronary artery disease.
Topics: Case-Control Studies; Citrulline; Coronary Artery Disease; Cross-Sectional Studies; Female; Humans; Male; Middle Aged; Radiography | 2015 |
Automated immunoassays for the autoantibodies to carbamylated or citrullinated telopeptides of type I and II collagens.
Topics: Adult; Arthritis, Rheumatoid; Autoantibodies; Automation; Citrulline; Collagen Type I; Collagen Type II; Female; Humans; Immunoassay; Luminescent Measurements; Male; Middle Aged; Peptide Fragments | 2015 |
Separate and overlapping specificities in rheumatoid arthritis antibodies binding to citrulline- and homocitrulline-containing peptides related to type I and II collagen telopeptides.
Topics: Adult; Aged; Arthritis, Rheumatoid; Autoantibodies; Citrulline; Cohort Studies; Collagen Type I; Collagen Type II; Female; Humans; Male; Middle Aged; Peptides; Protein Binding; Young Adult | 2015 |
Expression of citrulline and homocitrulline residues in the lungs of non-smokers and smokers: implications for autoimmunity in rheumatoid arthritis.
Topics: Arthritis, Rheumatoid; Autoimmunity; Citrulline; Female; Gene Expression Regulation; Humans; Lung; Male; Pulmonary Disease, Chronic Obstructive; Smoking | 2015 |
Homocitrulline: a new marker for differentiating acute from chronic renal failure.
Topics: Acute Kidney Injury; Aged; Biomarkers; Citrulline; Female; Humans; Kidney Failure, Chronic; Male; Middle Aged | 2016 |
Homocitrullination Is a Novel Histone H1 Epigenetic Mark Dependent on Aryl Hydrocarbon Receptor Recruitment of Carbamoyl Phosphate Synthase 1.
Topics: Animals; Base Sequence; Basic Helix-Loop-Helix Transcription Factors; Carbamoyl-Phosphate Synthase (Ammonia); Cells, Cultured; Chromatin; Citrulline; Epigenesis, Genetic; Female; Histones; Hydrolases; Lysine; Mice; Mice, Inbred C57BL; Nuclear Proteins; Polychlorinated Dibenzodioxins; Protein Binding; Protein Processing, Post-Translational; Protein Structure, Tertiary; Protein-Arginine Deiminases; Receptors, Aryl Hydrocarbon; Response Elements; Transcriptional Activation | 2015 |
Role of Anti-Carbamylated Protein Antibodies Compared to Anti-Citrullinated Protein Antibodies in Indigenous North Americans With Rheumatoid Arthritis, Their First-Degree Relatives, and Healthy Controls.
Topics: Adult; Arthritis, Rheumatoid; Autoantibodies; Citrulline; Female; Humans; Indians, North American; Male; Middle Aged; Peptides, Cyclic; Rheumatoid Factor | 2016 |
Ureido group-specific antibodies are induced in rabbits immunized with citrulline- or homocitrulline-containing antigens.
Topics: Animals; Antibody Specificity; Arthritis, Rheumatoid; Autoantibodies; Autoantigens; Carrier Proteins; Citrulline; Collagen; Disease Models, Animal; Immunization; Peptides; Protein Binding; Rabbits | 2016 |
Ornithine and Homocitrulline Impair Mitochondrial Function, Decrease Antioxidant Defenses and Induce Cell Death in Menadione-Stressed Rat Cortical Astrocytes: Potential Mechanisms of Neurological Dysfunction in HHH Syndrome.
Topics: Amino Acid Transport Systems, Basic; Animals; Antioxidants; Astrocytes; Cell Death; Citrulline; Hyperammonemia; Male; Mitochondria; Ornithine; Rats, Wistar; Tumor Necrosis Factor-alpha; Urea Cycle Disorders, Inborn | 2016 |
Homocitrulline as marker of protein carbamylation in hemodialyzed patients.
Topics: Biomarkers; Carbamates; Citrulline; Hemoglobin A; Humans; Longitudinal Studies; Renal Dialysis; Renal Insufficiency, Chronic | 2016 |
Antimodified protein antibody response pattern influences the risk for disease relapse in patients with rheumatoid arthritis tapering disease modifying antirheumatic drugs.
Topics: Acetates; Acetylation; Antirheumatic Agents; Arthritis, Rheumatoid; Autoantibodies; Carbamates; Citrulline; Humans; Logistic Models; Lysine; Multivariate Analysis; Ornithine; Peptides; Peptides, Cyclic; Prognosis; Randomized Controlled Trials as Topic; Recurrence; Vimentin | 2017 |
Eosinophil Peroxidase Catalyzed Protein Carbamylation Participates in Asthma.
Topics: A549 Cells; Animals; Asthma; Citrulline; Eosinophil Peroxidase; Eosinophils; Humans; Interferon-gamma; Interleukin-13; Mice; Protein Processing, Post-Translational; Th2 Cells; Transforming Growth Factor beta | 2016 |
Rheumatoid arthritis antigens homocitrulline and citrulline are generated by local myeloperoxidase and peptidyl arginine deiminases 2, 3 and 4 in rheumatoid nodule and synovial tissue.
Topics: Aged; Arthritis, Rheumatoid; Autoantigens; Blotting, Western; Chromatography, High Pressure Liquid; Citrulline; Female; Humans; Hydrolases; Immunohistochemistry; Male; Middle Aged; Peroxidase; Protein-Arginine Deiminase Type 2; Protein-Arginine Deiminases | 2016 |
Cyanate-Impaired Angiogenesis: Association With Poor Coronary Collateral Growth in Patients With Stable Angina and Chronic Total Occlusion.
Topics: Administration, Oral; Aged; Analysis of Variance; Angina, Stable; Angiogenesis Inducing Agents; Animals; Cells, Cultured; Chronic Disease; Citrulline; Collateral Circulation; Coronary Angiography; Coronary Occlusion; Cyanates; Endothelium, Vascular; Female; Hindlimb; Humans; Ischemia; Male; Mice, Inbred C57BL; Vascular Endothelial Growth Factor Receptor-2 | 2016 |
Carbamylation of vimentin is inducible by smoking and represents an independent autoantigen in rheumatoid arthritis.
Topics: Animals; Arthritis, Rheumatoid; Autoantibodies; Autoantigens; Carbamates; Case-Control Studies; Citrulline; Cross Reactions; Enzyme-Linked Immunosorbent Assay; Humans; Immunoblotting; Immunoglobulin G; Mice; Nicotiana; Protein Isoforms; Rabbits; Smoke; Smoking; Vimentin | 2017 |
Drastic decrease of global l-arginine bioavailability during orthotopic liver transplantation: A matter of ATP deficiency of the graft?
Topics: Adenosine Triphosphate; Arginine; Biological Availability; Citrulline; Humans; Liver Transplantation; Ornithine | 2018 |
Identification of carbamylated alpha 1 anti-trypsin (A1AT) as an antigenic target of anti-CarP antibodies in patients with rheumatoid arthritis.
Topics: alpha 1-Antitrypsin; Arthralgia; Arthritis, Rheumatoid; Autoantibodies; Autoantigens; Chromatography, Ion Exchange; Citrulline; Computational Biology; Enzyme-Linked Immunosorbent Assay; Humans; Mass Spectrometry; Peptide Fragments; Protein Conformation; Protein Processing, Post-Translational; Synovial Membrane | 2017 |
Brief Report: Anti-Carbamylated Protein Antibodies in Rheumatoid Arthritis Patients Are Reactive With Specific Epitopes of the Human Fibrinogen β-Chain.
Topics: Arthritis, Rheumatoid; Autoantibodies; Carbamates; Chromatography, Liquid; Citrulline; Electrophoresis, Polyacrylamide Gel; Enzyme-Linked Immunosorbent Assay; Epitopes; Fibrinogen; Humans; Immunoblotting; Mass Spectrometry | 2017 |
Impact of fibrinogen carbamylation on fibrin clot formation and stability.
Topics: Blood Coagulation; Chemotaxis, Leukocyte; Citrulline; Cyanates; Factor XIIIa; Fibrin; Fibrinogen; Fibrinolysis; Fibrinopeptide A; Humans; Kinetics; Neutrophils; Polymerization; Protein Conformation; Protein Processing, Post-Translational; Protein Stability; Renal Dialysis; Renal Insufficiency; Structure-Activity Relationship; Thrombin; Urea | 2017 |
Breach of autoreactive B cell tolerance by post-translationally modified proteins.
Topics: Animals; Arthritis, Experimental; Arthritis, Rheumatoid; Autoantibodies; Autoantigens; B-Lymphocytes; Carbamates; Case-Control Studies; Citrulline; Cross Reactions; Disease Models, Animal; Humans; Mass Spectrometry; Mice; Protein Processing, Post-Translational; Self Tolerance; Synovial Membrane | 2017 |
Hydroxyhomocitrulline Is a Collagen-Specific Carbamylation Mark that Affects Cross-link Formation.
Topics: Aging; Animals; Biomechanical Phenomena; Citrulline; Collagen; Humans; Mice; Organ Specificity; Protein Processing, Post-Translational; Renal Dialysis; Urea | 2017 |
Metabolomic Alterations Associated with Cause of CKD.
Topics: Adult; Citrulline; Deoxyglucose; Female; Glomerulonephritis; Guanosine; Hippurates; Homovanillic Acid; Humans; Kynurenic Acid; Male; Metabolome; Middle Aged; Palmitic Acids; Polycystic Kidney Diseases; Randomized Controlled Trials as Topic; Renal Insufficiency, Chronic | 2017 |
Metabolites related to renal function, immune activation, and carbamylation are associated with muscle composition in older adults.
Topics: Aged; Aging; Biomarkers; Blood Urea Nitrogen; Citrulline; Creatinine; Cross-Sectional Studies; Female; Humans; Insulin Resistance; Linear Models; Male; Metabolomics; Multivariate Analysis; Muscle, Skeletal; Muscular Atrophy; Tomography, X-Ray Computed | 2017 |
Relatedness of Antibodies to Peptides Containing Homocitrulline or Citrulline in Patients with Rheumatoid Arthritis.
Topics: Adult; Aged; Aged, 80 and over; Analysis of Variance; Antibody Affinity; Arthritis, Psoriatic; Arthritis, Rheumatoid; Autoantibodies; Citrulline; Enzyme-Linked Immunosorbent Assay; Epitopes; Female; Healthy Volunteers; Humans; Immunoglobulin G; Lupus Erythematosus, Systemic; Male; Middle Aged; Peptides, Cyclic; Statistics, Nonparametric; Young Adult | 2018 |
Immune responses to peptides containing homocitrulline or citrulline in the DR4-transgenic mouse model of rheumatoid arthritis.
Topics: Animals; Anti-Citrullinated Protein Antibodies; Arthritis, Rheumatoid; Citrulline; Disease Models, Animal; Epitopes; Female; Histocompatibility Antigens Class II; Humans; Immunity, Humoral; Immunization; Male; Mice; Mice, Inbred C57BL; Mice, Transgenic; Peptides | 2018 |
Myeloperoxidase-catalyzed oxidation of cyanide to cyanate: A potential carbamylation route involved in the formation of atherosclerotic plaques?
Topics: Animals; Citrulline; Cyanates; Cyanides; Humans; Mice; Mice, Knockout; Oxidation-Reduction; Peroxidase; Plaque, Atherosclerotic; Protein Carbamylation | 2018 |
Measurement of Homocitrulline, A Carbamylation-derived Product, in Serum and Tissues by LC-MS/MS.
Topics: Animals; Atherosclerosis; Citrulline; Humans; Protein Carbamylation; Renal Insufficiency, Chronic | 2018 |
Antibodies and B cells recognising citrullinated proteins display a broad cross-reactivity towards other post-translational modifications.
Topics: Acetylation; Aged; Anti-Citrullinated Protein Antibodies; Arthritis, Rheumatoid; Autoantibodies; B-Lymphocytes; Citrullination; Citrulline; Cross Reactions; Female; Humans; Immunoglobulin G; Male; Middle Aged; Protein Carbamylation; Protein Processing, Post-Translational; Receptors, Antigen, B-Cell | 2020 |
Quantification of the Effect of Citrulline and Homocitrulline Residues on the Collision-Induced Fragmentation of Peptides.
Topics: Chromatography, High Pressure Liquid; Citrulline; Peptides; Protein Carbamylation; Tandem Mass Spectrometry | 2020 |
Homocitrullination of lysine residues mediated by myeloid-derived suppressor cells in the tumor environment is a target for cancer immunotherapy.
Topics: Animals; Cell Line, Tumor; Citrulline; Humans; Immunotherapy; Lysine; Mice; Myeloid-Derived Suppressor Cells; Tumor Microenvironment | 2021 |