tiletamine hydrochloride has been researched along with nitisinone in 216 studies
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 0 (0.00) | 18.7374 |
| 1990's | 25 (11.57) | 18.2507 |
| 2000's | 51 (23.61) | 29.6817 |
| 2010's | 112 (51.85) | 24.3611 |
| 2020's | 28 (12.96) | 2.80 |
| Authors | Studies |
|---|---|
| Hjalmarson, O; Holme, E; Lindstedt, S; Lock, EA; Strandvik, B | 1 |
| al-Dhalimy, M; Finegold, M; Grompe, M; Kennaway, NG; Lindstedt, S; Ou, CN; Papaconstantinou, J; Torres-Ramos, CA | 1 |
| Bird, S; Collins, JE; Miller, NJ; Rice-Evans, CA | 1 |
| Auton, TR; Ellis, MK; Gowans, LA; Lock, EA; Provan, WM; Smith, LL; Whitfield, AC | 1 |
| Heubi, JE | 1 |
| Brett, EM; Clayton, PT; Gibbs, TC; Holme, E; Lindstedt, S; Payan, J | 1 |
| Al-Dhalimy, M; Brantly, M; Finegold, M; Grompe, M; Ou, CN; Overturf, K; Tanguay, R | 1 |
| Mitchell, GA | 1 |
| Bentkowski, Z; Holme, E; Lindstedt, S; Pronicka, E; Rowinska, E; Zawadzki, J | 1 |
| Holme, E; Lindstedt, S | 3 |
| Paradis, K | 1 |
| Ellis, MK; Gaskin, P; Lock, EA; Mutter, LC; Prisbylla, MP; Provan, WM; Robinson, M; Smith, LL | 1 |
| Hosszú, E; Kálmánchey, R; Klujber, V; Sallai, A; Szönyi, L | 1 |
| Al-Dhalimy, M; Finegold, M; Grompe, M; Manning, K; Ou, CN; Overturf, K | 1 |
| Ellis, MK; Gaskin, P; Lock, EA; Provan, WM; Robinson, M; Smith, LL | 1 |
| Anikster, Y; Gahl, WA; Nyhan, WL | 1 |
| Auton, TR; Ellis, MK; Gaskin, P; Lee, DL; Lock, EA; Mutter, LC; Prisbylla, MP; Provan, WM; Robinson, M; Smith, LL | 1 |
| al-Dhalimy, M; Finegold, M; Grompe, M; Overturf, K | 2 |
| Maeda, Y; Oda, K; Suzuki, T; Suzuki, Y; Yoshikawa, Y | 1 |
| Barkaoui, E; Bernard, O; Debray, D; Habès, D; Ogier, H | 1 |
| de Kremer Dodelson, R; Depetris-Boldini, C; Galetto, R; Videla, MP | 1 |
| Holme, E; Lambruschini, N; Lindstedt, S; Mas, A; Ros, J; Vilaseca, MA | 1 |
| Astner, L; Bielenstein, M; Ekberg, S | 1 |
| Sass, JO; Skladal, D | 1 |
| Ellis, MK; Gaskin, P; Lock, EA; McLean Provan, W; Robinson, M; Smith, LL | 1 |
| Blum, D; Clercx, A; De Laet, C; Goyens, P; Vamos, E; Vanden Eijnden, S | 1 |
| Heikinheimo, M; Pitkänen, ST; Salo, MK | 1 |
| González Pascual, E; Holme, E; Lambruschini Ferri, N; Mas Comas, A; Ros Viladoms, J; Vilaseca Buscà, MA | 1 |
| Eksborg, S; Hall, MG; Lumholtz, B; Provan, WM; Wilks, MF | 1 |
| Schwetz, BA | 1 |
| Ahmad, S; Lueder, GT; Teckman, JH | 1 |
| Hanauske-Abel, HM; Levy, J; Newfield, RS; Popowicz, A; Remotti, H | 1 |
| Anderson, PD; Anikster, Y; Bernardini, I; Fitzpatrick, DL; Gahl, WA; Gerber, LH; Huizing, M; Introne, WJ; Murphey, MD; Perry, MB; Phornphutkul, C | 1 |
| Lorenzini, S; Mannoni, A; Selvi, E | 1 |
| Lock, EA; Smith, LL | 1 |
| Bodamer, OA; Crone, J; Holme, E; Huber, WD; Möslinger, D; Schima, W; Stöckler Ipsiroglu, S | 1 |
| Gissen, P; McKiernan, PJ; Preece, MA; Willshaw, HA | 1 |
| Dalton, TP; Dieter, MZ; Freshwater, SL; Miller, ML; Nebert, DW; Shertzer, HG | 1 |
| Kavana, M; Moran, GR | 1 |
| Berger, R; Jacobs, SM; Klomp, LW; Koornneef, LP; Luijerink, MC; van Beurden, EA; van den Berg, IE | 1 |
| Yang, DY | 1 |
| Al-Dhalimy, M; Finegold, M; Grompe, M; Groopman, J; Iordanov, MS; Ou, CN; Ryabinina, O; van Den Berg, IE; Vogel, A | 1 |
| Brownlee, JM; Harrison, DH; Johnson-Winters, K; Moran, GR | 1 |
| Lock, EA; Williams, RE | 1 |
| Jaffe, IA; Keller, JM; Macaulay, W; Nercessian, OA | 1 |
| Berger, R; Grompe, M; Jacobs, SM; Klomp, LW; Luijerink, MC; Malingré, HE; van Beurden, EA; van den Berg, IE | 1 |
| Joshi, SN; Venugopalan, P | 1 |
| Felitsyn, NM; Henderson, GN; James, MO; Stacpoole, PW | 1 |
| André, N; Jubin, V; Ovaert, C; Roquelaure, B | 1 |
| Al Amoudi, M; Al-Ahaidib, LY; Al-Dbaas, A; Al-Dirbashi, OY; Al-Hassnan, Z; Al-Owain, M; Al-Sayed, MM; Ni Luanaigh, M; Rahbeeni, Z; Rashed, MS | 1 |
| Bernardini, I; Gahl, WA; Gerber, LH; Kaiser-Kupfer, MI; O'Brien, K; Perry, MB; Rubin, BI; Sebring, N; Suwannarat, P; Tsilou, E | 1 |
| Kobrinsky, NL; Sjolander, DE | 1 |
| Bergeron, A; Jorquera, R; Orejuela, D; Tanguay, RM | 1 |
| Batista, WL; Costa de Oliveira, R; da Silva Ferreira, ME; da Silva, VS; de Bragança Pereira, CA; de Souza Bernardes, LA; dos Reis Marques, E; Goldman, GH; Goldman, MH; Leite, DB; Nobrega, FG; Nóbrega, MP; Nunes, LR; Puccia, R; Ribeiro, DC; Travassos, LR; Yang, DY | 1 |
| Berger, R; Jacobs, SM; Klomp, LW; van Beurden, DH; van den Berg, IE | 1 |
| Ellis, M; Gaskin, P; Lock, EA; Provan, WM; Smith, LL | 1 |
| Finegold, M; Jorquera, R; Langlois, C; Shroads, AL; Stacpoole, PW; Tanguay, RM | 1 |
| Arora, N; Kelly, DA; McKiernan, PJ; Stumper, O; Wright, J | 1 |
| McKiernan, PJ | 1 |
| Bijleveld, CM; Koelink, CJ; van den Heuvel-Eibrink, MM; van der Ploeg, A; van Hasselt, P; van Spronsen, FJ; Wijburg, FA | 1 |
| Cohen, AF; van Bronswijk, H | 1 |
| Bergeron, A; Finegold, MJ; Jorquera, R; Langlois, C; Orejuela, D; Rhead, WJ; Tanguay, RM | 1 |
| Bergeron, A; Finegold, MJ; Jorquera, R; Orejuela, D; Tanguay, RM | 1 |
| Bernard, O; de Baulny, HO; Dobbelaere, D; Guffon, N; Masurel-Paulet, A; Poggi-Bach, J; Rolland, MO; Sarles, J; Touati, G | 1 |
| Baumann, U; Santra, S | 1 |
| Ketteler, K; Mayatepek, E; Perot, C; Schiff, M; Schlump, JU; Spiekerkoetter, U; Wendel, U | 1 |
| Hulton, SA; McKiernan, PJ; Preece, MA; Santra, S | 1 |
| Finegold, M; Grompe, M; Lee, AY; Rothfuss, A; Sharma, AD; Vogel, A; Wang, Z; Willenbring, H | 1 |
| Buitrago-Molina, LE; Cañón, JM; Finegold, M; Geffers, R; Grompe, M; Lamlé, J; Manns, MP; Marhenke, S; Sporn, M; Vogel, A; Yamamoto, M | 1 |
| Gradowska, W; Gryff-Keller, A; Lamparska, D; Szczeciński, P | 1 |
| Herebian, D; Lamshöft, M; Laryea, M; Mayatepek, E; Spiekerkötter, U; Thimm, E | 1 |
| Al-Dirbashi, O; El-Hawary, M; El-Karaksy, H; El-Koofy, N; El-Raziky, M; El-Sayed, R; Rashed, M | 1 |
| Aktuğlu-Zeybek, AC; Cansever, MS; Erim, FB | 1 |
| Herebian, D; Lamshöft, M; Mayatepek, E; Spiekerkoetter, U | 1 |
| Dahlke, MH; Doenecke, A; Eggenhofer, E; Geissler, EK; Piso, P; Popp, FC; Renner, P; Schlitt, HJ; Slowik, P | 1 |
| Callea, F; Castellano, A; Dionisi-Vici, C; Francalanci, P; Holme, E; Jenkner, A; Nobili, V | 1 |
| Bates, J; Brownlee, JM; Heinz, B; Moran, GR | 1 |
| Das, AM; Demirkol, M; Gökcay, G; Janzen, N; Ozer, I; Peter, M; Sander, J; Sander, S; Terhardt, M | 1 |
| Assmann, B; Herebian, D; Klee, D; Mayatepek, E; Spiekerkoetter, U; Thimm, E | 1 |
| Anolik, R; Pomeranz, MK; Yancovitz, M | 1 |
| Aldámiz-Echevarría, L; Andrade, F; Lage, S; Prieto, JA | 1 |
| Bernardini, I; Bryant, J; Gahl, WA; Introne, WJ; Kayser, MA; Moylan, E; O'Brien, KE; Perry, MB; Reynolds, JC; Sachdev, V; Suwannarat, P; Troendle, J; Tsilou, E | 1 |
| El-Hennawy, A; El-Karaksy, H; El-Kiki, H; El-Koofy, N; El-Raziky, M; El-Sayed, R; Fahmy, M; Mohsen, N; Rashed, MS | 1 |
| Cox, TF; Ranganath, L | 1 |
| Carton, D; Dan, B; De Laet, C; François, B; Goyens, PJ; Jaeken, J; Munoz, VT; Sokal, EM | 1 |
| Cox, TF; Ranganath, LR | 1 |
| Aldámiz-Echevarría, L; Couce, ML; Dalmau, J; del Toro, M; Pintos-Morell, G | 1 |
| Bleidissel, M; Cantz, T; Gentile, L; Greber, B; Han, DW; Liu, N; Ott, M; Rittelmeyer, I; Rudolph, C; Schambach, A; Schöler, HR; Sgodda, M; Sharma, AD; Steinemann, D; Wu, G; Zaehres, H | 1 |
| Cox, TM | 1 |
| Manga, P; Orlow, SJ | 1 |
| Adams, DR; Alur, RP; Bernardini, IM; Brilliant, MH; Brooks, BP; Chan, CC; Dolinska, MB; Gahl, WA; Onojafe, IF; Sergeev, YV; Simeonov, DR; Zhang, J | 1 |
| Herebian, D; Kamp, G; Klee, D; Mayatepek, E; Molke, B; Richter-Werkle, R; Spiekerkoetter, U; Thimm, E | 1 |
| Laidman, J | 1 |
| Boenzi, S; Della Bona, ML; Dionisi-Vici, C; la Marca, G; Malvagia, S; Martinelli, D; Materazzi, S | 1 |
| Herebian, D; Schlune, A; Spiekerkoetter, U; Thimm, E | 1 |
| Bartlett, DC; Holme, E; Lloyd, C; McKiernan, PJ; Newsome, PN; Preece, MA | 1 |
| Claerhout, I; Jaeken, J; Kestelyn, P; Schauwvlieghe, PP | 1 |
| Davit-Spraul, A; Poggi-Bach, J; Romdhane, H | 1 |
| McKiernan, P | 1 |
| Arias, C; Cabello, JF; Castro, G; Cornejo, V; de la Parra, A; Fernández, E; Raimann, E | 1 |
| Alvarez, F; Bussières, JF; Chevalier, I; Dallaire, L; Dubois, J; Faucher, F; Fenyves, D; Goodyer, P; Grenier, A; Holme, E; Laframboise, R; Lambert, M; Larochelle, J; Lindstedt, S; Maranda, B; Melançon, S; Merouani, A; Mitchell, GA; Mitchell, J; Parizeault, G; Pelletier, L; Phan, V; Rinaldo, P; Scott, CR; Scriver, C | 1 |
| van der Lelij, A; Visser, G; Wisse, RP; Wittebol-Post, D | 1 |
| Benoist, JF; Davit-Spraul, A; Garcia Segarra, N; Grenèche, MO; Imbard, A; Ogier de Baulny, H; Roche, S | 1 |
| de Baulny, HO; de Laet, C; Dionisi-Vici, C; Leonard, JV; McKiernan, P; Mitchell, G; Monti, L; Pintos-Morell, G; Spiekerkötter, U | 1 |
| Gallagher, JA; Jarvis, JC; Keenan, CM; Preston, AJ; Ranganath, LR; Sutherland, H; Taylor, AM; Williams, DP; Wilson, PJ; Wlodarski, B | 1 |
| Kassel, R; Rudnick, DA; Sprietsma, L | 1 |
| Al Jurayyan, NA; Al-Jarallah, AS; Al-Nemri, A; Babiker, A; Hasanato, R; Kambal, MA; Mohamed, S | 1 |
| Bendadi, F; de Koning, TJ; de Sain, MG; Prinsen, HC; Sinnema, G; van Hasselt, PM; van Spronsen, FJ; Verhoeven-Duif, N; Visser, G | 1 |
| Arning, E; Bottiglieri, T; Gibson, KM; Grompe, M; Harding, CO; Winn, SR | 1 |
| Bartlett, DC; Lloyd, C; McKiernan, PJ; Newsome, PN | 1 |
| Janovszky, A; Karg, E; László, A; Rózsa, M; Sallay, E; Tiszlavicz, L; Túri, S; Ugarte, M; Várkonyi, A; Wittmann, G | 1 |
| Bardy, SL; Ketelboeter, LM; L Bardy, S; M Ketelboeter, L; Potharla, VY; Y Potharla, V | 1 |
| Qi, Z; Sun, R; Tian, Z; Wang, X; Wei, H | 1 |
| Chapchap, P; Feier, FH; Fonseca, EA; Leite, KM; Miura, IK; Porta, A; Porta, G; Pugliese, R; Seda Neto, J | 1 |
| Aldamiz-Echevarria, LJ; Bliksrud, YT; Brunner-Krainz, M; Cocho de Juan, JA; Couce Pico, ML; Das, AM; de Baulny, HO; de Laet, C; Dionisi-Vici, C; Endig, J; Ernst, S; Freisinger, P; Gautschi, M; Gokcay, G; Hochuli, M; Jordan, J; Lotz-Havla, AS; Maiorana, A; Mandel, H; Mayorandan, S; McKiernan, P; Meyer, U; Moeslinger, D; Morlot, S; Sander, J; Santer, R; Scholl-Bürgi, S; Segarra, NG; Spiekerkoetter, U; Thimm, E; van Spronsen, F; Vogel, A; Zeman, J | 1 |
| Boenzi, S; Di Ciommo, VM; Dionisi-Vici, C; Emma, F; Maiorana, A; Malamisura, M | 1 |
| Altay, S; Aydin, A; Cansever, S; Erkan, T; Kiykim, E; Soyucen, E; Zeybek, AC; Zubarioglu, T | 1 |
| Lock, E; Ranganath, LR; Timmis, O | 1 |
| Ayoob, H; Braconi, D; Briggs, MC; Bygott, H; Christensen, P; Cox, TF; Cronlund, A; Dutton, JJ; Fitzgerald, R; Gallagher, JA; Genovese, F; Hall, AK; Hughes, AT; Imrich, R; Jarvis, JC; Junestrand, C; Kullenberg, T; Laan, D; Le Quan Sang, KH; McCaffrey, J; Milan, AM; Nemethova, M; Olsson, B; Psarelli, EE; Ranganath, LR; Ross, G; Rovensky, J; Rudebeck, M; Santucci, A; Sireau, N; Svensson, L; Szamosi, J; Timmis, OG; van Kan, C; Zatkova, A | 1 |
| Chakrapani, A; McKiernan, PJ; Preece, MA | 1 |
| Christensen, P; Davison, AS; Dutton, JJ; Gallagher, JA; Hughes, AT; Milan, AM; Ranganath, LR; Ross, G | 1 |
| Barshop, BA; Gangoiti, JA; Gertsman, I; Nyhan, WL | 1 |
| Arnoux, JB; Brassier, A; de Lonlay, P; Dubois, S; Grisel, C; Job-Deslandre, C; Le Quan Sang, KH; Ranganath, L; Servais, A; Sireau, N; Wippf, J | 1 |
| Hadžić, N; Vara, R | 1 |
| Bukhari, M; Jackson, DJ; Mistry, JB; Taylor, AM | 1 |
| Gallagher, JA; Ranganath, LR; Timmis, OG | 1 |
| Gao, X; Li, L; Qi, Z; Sun, R; Tian, Z; Wang, X; Wei, H; Zeng, Z | 1 |
| Bardy, SL; Ketelboeter, LM | 1 |
| Davison, AS; Gallagher, JA; Milan, AM; Ranganath, LR | 1 |
| Iwai, M; Katashima, M; Minoura, H; Takahashi, H; Taniuchi, Y | 1 |
| Bernardini, G; Botta, M; Braconi, D; Dreassi, E; Geminiani, M; Laschi, M; Manetti, F; Marzocchi, B; Millucci, L; Santucci, A | 1 |
| Shah, F; Shah, I | 1 |
| Horoz, ÖÖ; İncecik, F; Kör, D; Öktem, M; Önenli Mungan, N; Sander, J; Yıldızdaş, D | 1 |
| Barnby, E; Coker, SB; Hillgartner, MA; Koenig, AE; MacGregor, GG; Moore, ME | 1 |
| Derks, G; Dunster, JL; Mistry, P; Salazar, JD; Ward, JP | 1 |
| Amiot, B; Chen, H; DeGrado, TR; Elgilani, F; Freeman, BL; Glorioso, J; Grompe, M; Harding, CO; Hickey, RD; Ho, CS; Ibrahim, SH; Ikeda, Y; Jiang, H; Lillegard, JB; Mao, SA; Marler, R; Nyberg, SL; O'Connor, MK; Peng, KW; Rinaldo, P; Russell, SJ; Suksanpaisan, L | 1 |
| Barzi, M; Bissig, KD; Bissig-Choisat, B; Borowiak, M; Elsea, SH; Hubert, L; Legras, X; Mi, T; Pankowicz, FP; Ravishankar, M; Sumazin, P; Sun, Q; Tomolonis, JA; Yang, D | 1 |
| Knispel, JD; Leventhal, JS; McNiff, JM; Yin, ES | 1 |
| Gokay, S; Kardas, F; Kendirci, M; Ustkoyuncu, PS | 1 |
| Peake, RW | 1 |
| Mannion, MA; Mayne, P; Monavari, AA; Smith, A | 1 |
| Curtis, S; Davison, AS; Devine, J; Gallagher, JA; Hughes, AT; Khedr, M; Milan, AM; Ranganath, LR; Usher, J | 1 |
| Morrow, G; Tanguay, RM | 1 |
| Angileri, F; Tanguay, RM; Vogel, A | 1 |
| Dubois, J; Halac, U; Mitchell, GA | 1 |
| Dionisi-Vici, C; Maiorana, A | 1 |
| Pennings, JP; van Ginkel, WG; van Spronsen, FJ | 1 |
| Huijbregts, SCJ; Jahja, R; van Ginkel, WG; van Spronsen, FJ | 1 |
| Endo, F; Ito, M; Nakamura, K; Shigematsu, Y | 1 |
| Aktuglu-Zeybek, AC; Cansever, MS; Kiykim, E | 1 |
| Lock, EA | 1 |
| Alvarez, F; Atkinson, S; Bouchard, M; Brunel-Guitton, C; Buhas, D; Bussières, JF; Dubois, J; Fenyves, D; Goodyer, P; Gosselin, M; Halac, U; Labbé, P; Laframboise, R; Maranda, B; Melançon, S; Merouani, A; Mitchell, GA; Mitchell, J; Parizeault, G; Pelletier, L; Phan, V; Turcotte, JF | 1 |
| Das, AM; Meyer, U; van Rijn, M; van Spronsen, FJ | 1 |
| Mitchell, GA; Yang, H | 1 |
| Chinsky, JM; Coakley, K; Ficicioglu, C; Grompe, M; Gucsavas-Calikoglu, M; Mitchell, G; Scott, CR; Singh, R; van Karnebeek, CDM; Waisbren, SE; Wasserstein, MP | 1 |
| Clarke, A; Fraser, H; Freeman, K; Geppert, J; Johnson, S; Stinton, C; Sutcliffe, P; Taylor-Phillips, S | 1 |
| Burgerhof, JGM; Daly, A; de Blaauw, P; Heiner-Fokkema, MR; Kienstra, NS; MacDonald, A; McKiernan, PJ; van Dam, E; van Ginkel, WG; van Reemst, HE; van Spronsen, FJ | 1 |
| Christensen, M; Lund, AM; Sundberg, J; Wibrand, F | 1 |
| Davison, AS; Devine, J; Gallagher, JA; Harrold, JA; Hughes, AT; Hughes, G; J C G, H; Khedr, M; Milan, AM; Norman, BP; Ranganath, LR; Usher, J | 1 |
| Barton, GJ; Briggs, M; Cox, TF; Daroszewska, A; Davison, AS; Devine, JM; Dillon, JP; Fisher, M; Gallagher, JA; Griffin, R; Harrold, J; Hughes, AT; Hughes, G; Jarvis, JC; Jones, A; Judd, S; Khedr, M; Loftus, N; McCormick, M; Milan, AM; Psarelli, EE; Ranganath, LR; Sireau, N; Taylor, S; Usher, JL; Vinjamuri, S; West, E | 1 |
| Abu-Asab, MS; Adams, DR; Aderemi, JO; Albert, JS; Alur, RP; Bernardini, IM; Brooks, BP; Chan, CC; Cogliati, T; Megan, LH; Melch, MG; Onojafe, IF | 1 |
| Amoano, K; Hayashi, A; Hirayama, M; Inoue, M; Kurihara, K; Sakurai, H; Toyoda, H; Uchida, K | 1 |
| Bröijersén, A; Dalén, P; Huledal, G; Kruse, M; Lindqvist, D; Olsson, B; Önnestam, K | 1 |
| Adam, D | 1 |
| Dong, JQ; Hao, GF; Lin, HY; Wang, DW; Wang, YX; Wu, JW; Yang, GF; Yang, JF; Yang, WC; Zhan, CG | 1 |
| Kang, WM; Ndikuryayo, F; Wu, FX; Yang, GF; Yang, WC | 1 |
| Davison, AS; Gallagher, JA; Hughes, AT; Jarvis, JC; Milan, AM; Norman, BP; Ranganath, LR; Roberts, NB; Ross, GA; Sutherland, H | 1 |
| Hann, G; Strauss, CE | 1 |
| Barchanska, H; Mrachacz, M; Rola, R; Szczepankiewicz, W | 1 |
| Balanco, JMF; Gabriel, HB; Katzin, AM; Kimura, EA; Sussmann, RAC; Verdaguer, IB | 1 |
| Aydogdu, S; Farajov, R; Iakobadze, Z; Karaca, CA; Kilic, M; Yilmaz, C | 1 |
| Curtis, SL; Gallagher, JA; Milan, AM; Norman, BP; Olsson, B; Ranganath, LR; Roberts, NB | 1 |
| Alqahtani, A; Blum, A; Feillet, F; Guéant, JL; Henn-Ménétré, S; Jeannesson, E; Merten, M; Renard, E; Sloboda, N; Wiedemann, A | 1 |
| Chen, F; Chen, J; Fu, Z; Hu, YP; Liu, Q; Song, S; Sun, Y; Wang, MJ; Yang, T; Zhao, L | 1 |
| Cox, TF; Davison, AS; Gallagher, JA; Hughes, AT; Khedr, M; Milan, AM; Psarelli, EE; Ranganath, LR; Rhodes, NP; Rovensky, J | 1 |
| Heiner-Fokkema, MR; Lubout, CMA; Rodenburg, IL; van der Klauw, MM; van Ginkel, WG; van Spronsen, FJ; van Vliet, K; Wolffenbuttel, BHR | 1 |
| Bou-Gharios, G; Gallagher, JA; Hughes, AT; Hughes, JH; Jarvis, JC; Judd, S; Milan, AM; Ranganath, LR; Sutherland, H; Wilson, PJM | 1 |
| Davison, AS; Gallagher, JA; Hughes, AT; Milan, AM; Ranganath, LR; Sireau, N | 1 |
| Aldámiz-Echevarría, L; Bueno, M; Couce, ML; Díaz-Fernández, C; García-Volpe, C; Gil, D; Hernández, T; López-Ruzafa, E; Martín-Hernández, E; Navas, V; Peña-Quintana, L; Pintos, G; Roca, I; Sánchez-Pintos, P; Sánchez-Valverde, F; Vitoria, I | 1 |
| Chelliah, Y; Chen, J; Cox, KH; de Groot, M; Kumar, V; Siepka, SM; Takahashi, JS; Tu, B; Yang, S | 1 |
| Harding, CO; Heiner-Fokkema, MR; Hollak, CEM; Rodenburg, IL; van Ginkel, WG; van Spronsen, FJ | 1 |
| Burgerhof, JGM; Daly, A; de Blaauw, P; Heiner-Fokkema, MR; Kienstra, NS; MacDonald, A; Rodenburg, IL; Santra, S; van de Krogt, J; van Ginkel, WG; van Reemst, HE; van Spronsen, FJ | 1 |
| Bierau, J; Cassiman, D; Daly, A; De Laet, C; Eyskens, F; Gissen, P; Goyens, PJ; Huijbregts, SCJ; Jahja, R; Jameson, EA; MacDonald, A; McKiernan, PJ; Morris, AAM; Mumford, N; Rahman, Y; Timmer, C; van Ginkel, WG; van Hasselt, PM; van Spronsen, FJ; van Vliet, K; Vara, R; Wilcox, G | 1 |
| De Bernardo, M; Guercio Nuzio, S; Mandato, C; Rosa, N; Vajro, P; Vitiello, L | 1 |
| Hong, F; Huang, X; Liu, C; Qian, G; Shu, Q; Tong, F; Wu, D; Yang, R; Zhang, T; Zhao, Z; Zhou, X | 1 |
| Cooper, MS; Davison, AS; Deutz, NEP; Fitzgerald, R; Gallagher, JA; Ghane, P; Hughes, AT; Jarvis, JC; Khedr, M; Markinson, L; Milan, AM; Norman, BP; Psarelli, EE; Ranganath, LR; Sutherland, H | 1 |
| Almuqbil, M; Chinsky, JM; Knoll, J | 1 |
| Äärelä, L; Hiltunen, P; Kurppa, K; Nevalainen, PI | 1 |
| Gavrilov, DK; Matern, D; Netzel, BC; Oglesbee, D; Pino, GB; Raymond, KM; Rinaldo, P; Schultz, MJ; Singh, RH; Smith, WE; Tortorelli, S | 1 |
| Häberle, J | 1 |
| Arnoux, JB; Braconi, D; Briggs, M; Bröijersén, A; Bygott, H; Cox, TF; Davison, AS; Dillon, JP; Fisher, M; FitzGerald, R; Gallagher, JA; Genovese, F; Glasova, H; Hall, AK; Hughes, AT; Hughes, JH; Imrich, R; Jarvis, JC; Khedr, M; Laan, D; Le Quan Sang, KH; Loftus, N; Luangrath, E; Lukáčová, O; Milan, AM; Mistry, A; Mlynáriková, V; Norman, BP; Olsson, B; Psarelli, EE; Ranganath, LR; Rhodes, NP; Rovenský, J; Rudebeck, M; Santucci, A; Scott, C; Sedláková, J; Shweihdi, E; Sireau, N; Stančík, R; Szamosi, J; Taylor, S; van Kan, C; Vinjamuri, S; Vrtíková, E; Webb, C; West, E; Záňová, E; Zatkova, A | 1 |
| Davidson, J; Gallagher, JA; Ranganath, LR; Vinjamuri, S | 1 |
| Branson, S; Casimir, G; Claes, P; Colemonts-Vroninks, H; De Kock, J; Goyens, P; Marcelis, L; Martens, GA; Neuckermans, J; Vanhaecke, T | 1 |
| Bärhold, F; Beblo, S; Bogovic, N; Dahl, SV; Das, AM; Dokoupil, K; Fekete, A; Fischer, A; Freisinger, P; Heddrich-Ellerbrok, M; Hochuli, M; Jörg-Streller, M; Korpel, V; Lahl, J; Lier, D; Mayorandan, S; Meyer, U; Möslinger, D; Neugebauer, AK; Och, U; Rohde, C; Rosenbaum-Fabian, S; Thimm, EM; van Teeffelen-Heithoff, A | 1 |
| Gallagher, JA; Khedr, M; Ranganath, LR; Vinjamuri, S | 1 |
| Acosta-Serrano, Á; Barribeau, SM; Casas-Sánchez, A; García Escude, N; Haines, LR; Ismail, HM; MacRae, JI; Masiga, D; Oliveira, PL; Owino Adung'a, V; Paine, MI; Quek, S; Rose, C; Silva Dos Santos, M; Sterkel, M; Vionette-Amaral, RJ; Wagstaff, S; Yakob, L | 1 |
| Couce, ML; Das, AM; de Laet, C; Dionisi-Vici, C; Lund, AM; Rudebeck, M; Schiff, M; Spada, M; Sparve, E; Spiekerkoetter, U; Szamosi, J; Vara, R | 1 |
| Introne, WJ | 1 |
| Fisher, MF; Heseltine, T; Khedr, M; Ranganath, LR | 1 |
| Babiuch, M; Barchanska, H; Płonka, J | 1 |
| Heiner-Fokkema, MR; van Spronsen, FJ; Wolffenbuttel, BHR | 1 |
| Hakeem, A; Jalan, A; Menon, J; Reddy, MS; Rela, M; Shanmugam, N; Valamparampil, JJ; Vij, M | 1 |
| Höller, A; Janzen, N; Jörg-Streller, M; Karall, D; Ramoni, A; Ramoser, G; Scholl-Bürgi, S; Zöggeler, T | 1 |
| Aktuglu Zeybek, AC; Cansever, MS; Erel, O; Iscan, HZ; Kiykim, E; Neselioglu, S; Zubarioglu, T | 1 |
| Barchańska, H; Danek, M; Korytkowska-Wałach, A | 1 |
| Arslan Gulten, Z; Arslan, N; Aydogan, A; Bahceci, H; Eroglu Erkmen, S; Karalar Pekuz, OK; Ozturk, T; Teke Kisa, P; Uysal, S; Yuce Inel, T | 1 |
| Balouch, F; Bhushan, S; Ee, L; Hodgkinson, P; Lampe, G; Lewindon, P; McGill, J; Noble, C | 1 |
| Arnoux, JB; Bou-Gharios, G; Davison, AS; Gallagher, JA; Hughes, AT; Imrich, R; M, K; Milan, AM; Norman, BP; Olsson, B; Ranganath, LR; Rudebeck, M | 1 |
| Gonzalez-Lamuño, D; Puente, N; Riancho, JA | 1 |
| Barchanska, H; Kostina-Bednarz, M; Nowak, P; Płonka, J | 1 |
32 review(s) available for tiletamine hydrochloride and nitisinone
| Article | Year |
|---|---|
Diagnosis and management of tyrosinemia type I.
Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Amino Acid Metabolism, Inborn Errors; Animals; Cyclohexanones; Disease Models, Animal; Enzyme Inhibitors; Genetic Therapy; Humans; Liver Transplantation; Nitrobenzoates; Phenotype; Tyrosine | 1995 |
Tyrosinemia: the Quebec experience.
Topics: Amino Acid Metabolism, Inborn Errors; Cyclohexanones; Enzyme Inhibitors; Humans; Infant; Liver Transplantation; Nitrobenzoates; Tyrosine | 1996 |
From toxicological problem to therapeutic use: the discovery of the mode of action of 2-(2-nitro-4-trifluoromethylbenzoyl)-1,3-cyclohexanedione (NTBC), its toxicology and development as a drug.
Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Amino Acid Metabolism, Inborn Errors; Animals; Cyclohexanones; Enzyme Inhibitors; Herbicides; Humans; Nitrobenzoates; Tyrosine | 1998 |
Tyrosinaemia type I and NTBC (2-(2-nitro-4-trifluoromethylbenzoyl)-1,3-cyclohexanedione).
Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Amino Acid Metabolism, Inborn Errors; Animals; Clinical Trials as Topic; Cyclohexanones; Enzyme Inhibitors; Humans; Nitrobenzoates; Tyrosine | 1998 |
Hereditary tyrosinaemia type I: from basics to progress in treatment.
Topics: Animals; Cyclohexanones; Enzyme Inhibitors; Humans; Liver Transplantation; Mice; Mutation; Nitrobenzoates; Pedigree; Tyrosine Transaminase; Tyrosinemias | 2000 |
Nontransplant treatment of tyrosinemia.
Topics: Age Factors; Animals; Carcinoma, Hepatocellular; Child, Preschool; Cyclohexanones; Enzyme Inhibitors; Humans; Infant; Liver Neoplasms; Nitrobenzoates; Risk Factors; Tyrosinemias | 2000 |
The role of mode of action studies in extrapolating to human risks in toxicology.
Topics: Animals; Carcinogenicity Tests; Carcinogens; Cyclohexanones; Guidelines as Topic; Half-Life; Humans; Nitrobenzoates; Risk Assessment; Tamoxifen; United States; United States Environmental Protection Agency | 2003 |
4-Hydroxyphenylpyruvate dioxygenase as a drug discovery target.
Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Cyclohexanones; Drug Design; Enzyme Inhibitors; Forecasting; Isoxazoles; Molecular Structure; Nitriles; Nitrobenzoates; Oxygenases; Sulfones; Tyrosinemias | 2003 |
New developments in ochronosis: review of the literature.
Topics: Alkaptonuria; Arthroplasty, Replacement; Cyclohexanones; Enzyme Inhibitors; Humans; Joint Diseases; Nitrobenzoates; Ochronosis | 2005 |
Experience of nitisinone for the pharmacological treatment of hereditary tyrosinaemia type 1.
Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Carcinoma, Hepatocellular; Child; Cyclohexanones; Enzyme Inhibitors; Humans; Liver Diseases; Liver Neoplasms; Monitoring, Physiologic; Nitrobenzoates; Tyrosinemias | 2008 |
Recommendations for the management of tyrosinaemia type 1.
Topics: Bone Density; Cyclohexanones; Female; Humans; Infant, Newborn; Liver Transplantation; Neonatal Screening; Nitrobenzoates; Pregnancy; Psychometrics; Tyrosinemias | 2013 |
The role of nitisinone in tyrosine pathway disorders.
Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Alkaptonuria; Animals; Biomedical Research; Cyclohexanones; Enzyme Inhibitors; Humans; Nitrobenzoates; Off-Label Use; Tyrosinemias | 2014 |
Old treatments for new insights and strategies: proposed management in adults and children with alkaptonuria.
Topics: Adult; Age Factors; Alkaptonuria; Antioxidants; Child; Cyclohexanones; Humans; Nitrobenzoates; Ochronosis; Phenylalanine; Tyrosine | 2015 |
Acute fatal metabolic complications in alkaptonuria.
Topics: Acute Disease; Alkaptonuria; Cyclohexanones; Erythrocytes; Homogentisate 1,2-Dioxygenase; Homogentisic Acid; Humans; Metabolic Diseases; Nitrobenzoates; Oxidation-Reduction | 2016 |
Biochemical and Clinical Aspects of Hereditary Tyrosinemia Type 1.
Topics: Cyclohexanones; Humans; Kidney; Liver; Liver Failure; Nitrobenzoates; Tyrosine; Tyrosinemias | 2017 |
Molecular Pathogenesis of Liver Injury in Hereditary Tyrosinemia 1.
Topics: Animals; Cyclohexanones; Humans; Hydrolases; Liver; Liver Diseases; Nitrobenzoates; Signal Transduction; Tyrosinemias | 2017 |
The Liver in Tyrosinemia Type I: Clinical Management and Course in Quebec.
Topics: Cyclohexanones; Humans; Hydrolases; Liver; Liver Diseases; Nitrobenzoates; Quebec; Tyrosinemias | 2017 |
NTBC and Correction of Renal Dysfunction.
Topics: Animals; Cyclohexanones; Humans; Kidney; Kidney Diseases; Liver; Liver Diseases; Nitrobenzoates; Tyrosinemias | 2017 |
Liver Cancer in Tyrosinemia Type 1.
Topics: Biomarkers, Tumor; Carcinoma, Hepatocellular; Cyclohexanones; Early Detection of Cancer; Humans; Liver Neoplasms; Nitrobenzoates; Tyrosinemias | 2017 |
Neurological and Neuropsychological Problems in Tyrosinemia Type I Patients.
Topics: Cyclohexanones; Humans; Nervous System Diseases; Nitrobenzoates; Phenylalanine; Tyrosine; Tyrosinemias | 2017 |
Hereditary Tyrosinemia Type 1 in Turkey.
Topics: Cyclohexanones; Humans; Hydrolases; Liver; Liver Diseases; Nitrobenzoates; Turkey; Tyrosine; Tyrosinemias | 2017 |
From Weed Killer to Wonder Drug.
Topics: Animals; Biological Products; Cyclohexanones; Enzyme Inhibitors; Humans; Liver; Nitrobenzoates; Tyrosine; Tyrosinemias | 2017 |
The Québec NTBC Study.
Topics: Cyclohexanones; Enzyme Inhibitors; Heptanoates; Humans; Infant, Newborn; Liver Diseases; Liver Transplantation; Neonatal Screening; Nitrobenzoates; Quebec; Tyrosinemias | 2017 |
Dietary Considerations in Tyrosinemia Type I.
Topics: Cyclohexanones; Diet; Humans; Nitrobenzoates; Phenylalanine; Tyrosine; Tyrosinemias | 2017 |
Remaining Challenges in the Treatment of Tyrosinemia from the Clinician's Viewpoint.
Topics: Animals; Cyclohexanones; Diet; Diet Therapy; Humans; Infant, Newborn; Kidney Diseases; Liver Failure; Liver Neoplasms; Liver Transplantation; Neonatal Screening; Nitrobenzoates; Tyrosinemias | 2017 |
Diagnosis and treatment of tyrosinemia type I: a US and Canadian consensus group review and recommendations.
Topics: Canada; Cyclohexanones; Diet Therapy; Disease Management; Female; Genetic Counseling; Genotype; Humans; Infant, Newborn; Liver Transplantation; Medication Adherence; Neonatal Screening; Nitrobenzoates; Phenotype; Pregnancy; Pregnancy Complications; Tyrosinemias; United States | 2017 |
Evaluation of pre-symptomatic nitisinone treatment on long-term outcomes in Tyrosinemia type 1 patients: a systematic review.
Topics: Cyclohexanones; Humans; Infant, Newborn; Neonatal Screening; Nitrobenzoates; Tyrosinemias | 2017 |
Alkaptonuria - Many questions answered, further challenges beckon.
Topics: Alkaptonuria; Animals; Cyclohexanones; Disease Models, Animal; Enzyme Inhibitors; Homogentisic Acid; Humans; Mice; Nitrobenzoates; Tyrosine | 2020 |
Long-Term Outcomes and Practical Considerations in the Pharmacological Management of Tyrosinemia Type 1.
Topics: Cyclohexanones; Humans; Nitrobenzoates; Tyrosine; Tyrosinemias | 2019 |
Pediatric liver diseases and ocular changes: What hepatologists and ophthalmologists should know and share with each other.
Topics: Child; Cyclohexanones; Eye Diseases; Gastroenterologists; Glucocorticoids; Humans; Liver Diseases; Nitrobenzoates; Ophthalmologists; Pediatrics | 2020 |
Suitability of nitisinone for alkaptonuria.
Topics: Alkaptonuria; Cyclohexanones; Enzyme Inhibitors; Humans; Nitrobenzoates | 2020 |
Efficacy of Phenylalanine- and Tyrosine-Restricted Diet in Alkaptonuria Patients on Nitisinone Treatment: Case Series and Review of Literature.
Topics: Adult; Alkaptonuria; Cyclohexanones; Diet; Dietary Proteins; Female; Humans; Male; Middle Aged; Nitrobenzoates; Phenylalanine; Tyrosine | 2022 |
9 trial(s) available for tiletamine hydrochloride and nitisinone
| Article | Year |
|---|---|
Pharmacokinetics and pharmacodynamics of NTBC (2-(2-nitro-4-fluoromethylbenzoyl)-1,3-cyclohexanedione) and mesotrione, inhibitors of 4-hydroxyphenyl pyruvate dioxygenase (HPPD) following a single dose to healthy male volunteers.
Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Administration, Oral; Area Under Curve; Chemistry, Pharmaceutical; Cyclohexanones; Enzyme Inhibitors; Half-Life; Humans; Male; Nitrobenzoates; Therapeutic Equivalency; Tyrosine; Tyrosinemias | 2001 |
Ophthalmic follow-up of patients with tyrosinaemia type I on NTBC.
Topics: Child; Child, Preschool; Corneal Opacity; Cyclohexanones; Enzyme Inhibitors; Female; Follow-Up Studies; Humans; Infant; Infant, Newborn; Male; Nitrobenzoates; Tyrosine; Tyrosinemias | 2003 |
A 3-year randomized therapeutic trial of nitisinone in alkaptonuria.
Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Adult; Alkaptonuria; Cyclohexanones; Homogentisic Acid; Humans; Middle Aged; Nitrobenzoates; Prospective Studies; Tyrosine | 2011 |
Neuropsychological outcome of NTBC-treated patients with tyrosinaemia type 1.
Topics: Adolescent; Belgium; Child; Child, Preschool; Cognition Disorders; Cyclohexanones; Enzyme Inhibitors; Female; Follow-Up Studies; Humans; Intelligence; Intelligence Tests; Male; Neuropsychological Tests; Nitrobenzoates; Phenylalanine; Treatment Outcome; Tyrosine; Tyrosinemias | 2011 |
Suitability Of Nitisinone In Alkaptonuria 1 (SONIA 1): an international, multicentre, randomised, open-label, no-treatment controlled, parallel-group, dose-response study to investigate the effect of once daily nitisinone on 24-h urinary homogentisic acid
Topics: Adult; Alkaptonuria; Cyclohexanones; Dose-Response Relationship, Drug; Drug Administration Schedule; Enzyme Inhibitors; Female; Homogentisic Acid; Humans; Male; Middle Aged; Nitrobenzoates; Research Design; Tyrosine | 2016 |
Non randomized study on the potential of nitisinone to inhibit cytochrome P450 2C9, 2D6, 2E1 and the organic anion transporters OAT1 and OAT3 in healthy volunteers.
Topics: Adolescent; Adult; Area Under Curve; Cyclohexanones; Cytochrome P-450 CYP2C9; Cytochrome P-450 CYP2D6; Cytochrome P-450 CYP2E1; Enzyme Inhibitors; Female; Healthy Volunteers; Humans; Male; Middle Aged; Nitrobenzoates; Organic Anion Transport Protein 1; Organic Anion Transporters, Sodium-Independent; Substrate Specificity; Young Adult | 2019 |
Quantification of the flux of tyrosine pathway metabolites during nitisinone treatment of Alkaptonuria.
Topics: Adult; Alkaptonuria; Cyclohexanones; Female; Homogentisic Acid; Humans; Male; Middle Aged; Nitrobenzoates; Phenylalanine; Pigments, Biological; Tandem Mass Spectrometry; Tyrosine | 2019 |
Efficacy and safety of once-daily nitisinone for patients with alkaptonuria (SONIA 2): an international, multicentre, open-label, randomised controlled trial.
Topics: Adult; Aged; Alkaptonuria; Cyclohexanones; Drug Administration Schedule; Enzyme Inhibitors; Female; Homogentisic Acid; Humans; Internationality; Longitudinal Studies; Male; Middle Aged; Nitrobenzoates; Single-Blind Method; Treatment Outcome | 2020 |
Long-term safety and outcomes in hereditary tyrosinaemia type 1 with nitisinone treatment: a 15-year non-interventional, multicentre study.
Topics: Adolescent; Chemical and Drug Induced Liver Injury; Child; Child, Preschool; Cyclohexanones; Enzyme Inhibitors; Female; Follow-Up Studies; Humans; Infant; Infant, Newborn; Kidney Diseases; Longitudinal Studies; Male; Neonatal Screening; Nitrobenzoates; Prospective Studies; Time Factors; Treatment Outcome; Tyrosinemias | 2021 |
175 other study(ies) available for tiletamine hydrochloride and nitisinone
| Article | Year |
|---|---|
New treatment for tyrosinaemia.
Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Amino Acid Metabolism, Inborn Errors; Cyclohexanones; Humans; Infant; Kidney Diseases; Liver Diseases; Nitrobenzoates; Tyrosine | 1992 |
Treatment of hereditary tyrosinaemia type I by inhibition of 4-hydroxyphenylpyruvate dioxygenase.
Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Acetoacetates; alpha-Fetoproteins; Amino Acid Metabolism, Inborn Errors; Aminolevulinic Acid; Child; Child, Preschool; Cyclohexanones; Erythrocytes; Heptanoates; Humans; Hydroxybenzoates; Infant; Kidney Tubules; Liver; Nitrobenzoates; Phenylalanine; Phosphates; Porphobilinogen Synthase; Proteinuria; Tyrosine | 1992 |
Pharmacological correction of neonatal lethal hepatic dysfunction in a murine model of hereditary tyrosinaemia type I.
Topics: alpha-Fetoproteins; Amino Acid Metabolism, Inborn Errors; Amino Acids; Animals; Cyclohexanones; Disease Models, Animal; Enzyme Inhibitors; Female; Heptanoates; Humans; Hydrolases; Liver; Liver Diseases; Liver Neoplasms; Male; Mice; Mice, Inbred C57BL; Nitrobenzoates; Pancreas; RNA, Messenger; Tyrosine | 1995 |
Plasma antioxidant capacity in two cases of tyrosinaemia type 1: one case treated with NTBC.
Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Amino Acid Metabolism, Inborn Errors; Antioxidants; Bilirubin; Cyclohexanones; Enzyme Inhibitors; Fatal Outcome; Female; Humans; Infant; Infant, Newborn; Male; Nitrobenzoates; Serum Albumin; Tyrosine | 1995 |
Inhibition of 4-hydroxyphenylpyruvate dioxygenase by 2-(2-nitro-4-trifluoromethylbenzoyl)-cyclohexane-1,3-dione and 2-(2-chloro-4-methanesulfonylbenzoyl)-cyclohexane-1,3-dione.
Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Amino Acid Metabolism, Inborn Errors; Animals; Cyclohexanones; Liver; Magnetic Resonance Spectroscopy; Male; Mesylates; Nitrobenzoates; Phenylpyruvic Acids; Rats; Rats, Wistar; Tyrosine | 1995 |
Promising new treatment for type I tyrosinemia.
Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Amino Acid Metabolism, Inborn Errors; Child, Preschool; Cyclohexanones; Humans; Nitrobenzoates; Tyrosine | 1993 |
Peripheral neuropathy as the presenting feature of tyrosinaemia type I and effectively treated with an inhibitor of 4-hydroxyphenylpyruvate dioxygenase.
Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Amino Acid Metabolism, Inborn Errors; Child, Preschool; Cyclohexanones; Electromyography; Female; Humans; Neural Conduction; Nitrobenzoates; Peripheral Nervous System Diseases; Tyrosine | 1993 |
Hepatocytes corrected by gene therapy are selected in vivo in a murine model of hereditary tyrosinaemia type I.
Topics: Amino Acid Metabolism, Inborn Errors; Animals; Cell Count; Cell Transplantation; Cyclohexanones; Disease Models, Animal; Enzyme Inhibitors; Genetic Therapy; Humans; Hydrolases; Immunoenzyme Techniques; Liver; Liver Function Tests; Liver Neoplasms; Male; Mice; Mice, Inbred C57BL; Nitrobenzoates; Retroviridae; Tyrosine | 1996 |
Inhibition of 4-hydroxyphenylpyruvate dioxygenase by 2-(2-nitro-4-trifluoromethylbenzoyl)-cyclohexane-1,3-dione and 2-(2-chloro-4-methanesulfonylbenzoyl)-cyclohexane-1,3-dione.
Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Animals; Cyclohexanones; Enzyme Inhibitors; Humans; Lethal Dose 50; Metabolism, Inborn Errors; Nitrobenzoates; Phenylpropionates; Phenylpyruvic Acids; Rats; Tyrosine | 1996 |
Treatment of two children with hereditary tyrosinaemia type I and long-standing renal disease with a 4-hydroxyphenylpyruvate dioxygenase inhibitor (NTBC).
Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Amino Acid Metabolism, Inborn Errors; Child; Cyclohexanones; Enzyme Inhibitors; Female; Humans; Kidney Diseases; Male; Nitrobenzoates; Osteoporosis; Phosphates; Reference Values; Tyrosine; Uric Acid | 1996 |
Tissue distribution of 2-(2-nitro-4-trifluoromethylbenzoyl)cyclohexane-1-3-dione (NTBC): effect on enzymes involved in tyrosine catabolism and relevance to ocular toxicity in the rat.
Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Animals; Aqueous Humor; Cyclohexanones; Eye; Liver; Male; Nitrobenzoates; Rats; Rats, Wistar; Tissue Distribution; Tyrosine | 1996 |
[Late onset type I tyrosinemia].
Topics: alpha-Fetoproteins; Amino Acid Metabolism, Inborn Errors; Biopsy; Child, Preschool; Cyclohexanones; Enzyme Inhibitors; Female; Humans; Liver Cirrhosis; Nitrobenzoates; Palliative Care; Porphyrias; Tyrosine | 1997 |
Ex vivo hepatic gene therapy of a mouse model of Hereditary Tyrosinemia Type I.
Topics: Amino Acid Metabolism, Inborn Errors; Animals; Base Sequence; Cell Survival; Cell Transplantation; Cells, Cultured; Cyclohexanones; Disease Models, Animal; DNA, Complementary; Enzyme Inhibitors; Female; Genetic Diseases, Inborn; Genetic Therapy; Humans; Hydrolases; Liver; Male; Mice; Molecular Sequence Data; Nitrobenzoates; Tyrosine | 1998 |
The effect of a low-protein diet and dietary supplementation of threonine on tyrosine and 2-(2-nitro-4-trifluoromethylbenzoyl) cyclohexane-1,3-dione-induced corneal lesions, the extent of tyrosinemia, and the activity of enzymes involved in tyrosine catab
Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Animals; Aqueous Humor; Corneal Diseases; Cyclohexanones; Dietary Proteins; Enzyme Inhibitors; Kidney; Liver; Male; Methionine; Nitrobenzoates; Rats; Rats, Wistar; Threonine; Tyrosine | 1998 |
NTBC and alkaptonuria.
Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Alkaptonuria; Amino Acid Metabolism, Inborn Errors; Cyclohexanones; Enzyme Inhibitors; Humans; Nitrobenzoates; Tyrosine | 1998 |
Therapeutic trials in the murine model of hereditary tyrosinaemia type I: a progress report.
Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Amino Acid Metabolism, Inborn Errors; Animals; Cyclohexanones; Enzyme Inhibitors; Genetic Therapy; Humans; Hydrolases; Liver; Mice; Mice, Inbred C57BL; Mice, Knockout; Nitrobenzoates; Tyrosine | 1998 |
A novel therapeutic trial of homogentisic aciduria in a murine model of alkaptonuria.
Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Alkaptonuria; Animals; Cyclohexanones; Disease Models, Animal; Enzyme Inhibitors; Homogentisic Acid; Mice; Mice, Mutant Strains; Nitrobenzoates | 1999 |
[Favorable outcome of treatment with NTBC of acute liver insufficiency disclosing hereditary tyrosinemia type I].
Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Acute Disease; Amino Acid Metabolism, Inborn Errors; Aminolevulinic Acid; Cyclohexanones; Enzyme Inhibitors; Female; Follow-Up Studies; Heptanoates; Humans; Infant; Infant, Newborn; Liver Failure; Male; Methionine; Nitrobenzoates; Porphobilinogen Synthase; Treatment Outcome; Tyrosine | 1999 |
Increased excretion of coproporphyrin I in a patient with hereditary tyrosinaemia type I: relevant changes with NTBC treatment.
Topics: Amino Acid Metabolism, Inborn Errors; Child; Coproporphyrins; Cyclohexanones; Enzyme Inhibitors; Female; Genetic Diseases, Inborn; Humans; Hydrolases; Nitrobenzoates; Tyrosine | 1999 |
NTBC as palliative treatment in chronic tyrosinaemia type I.
Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Adolescent; Amino Acid Metabolism, Inborn Errors; Chronic Disease; Cyclohexanones; Fatal Outcome; Humans; Male; Nitrobenzoates; Tyrosine | 1999 |
Determination of 2-(2-nitro-4-trifluoromethylbenzoyl)-1,3-cyclohexanedione in plasma by direct injection into a coupled column liquid chromatographic system.
Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Animals; Chromatography, Liquid; Cyclohexanones; Enzyme Inhibitors; Nitrobenzoates; Rats; Reproducibility of Results; Sensitivity and Specificity; Spectrophotometry, Ultraviolet | 1999 |
Elevated plasma bile acid concentrations in two sisters with tyrosinaemia type I.
Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Bile Acids and Salts; Cyclohexanones; Enzyme Inhibitors; Female; Humans; Infant; Infant, Newborn; Nitrobenzoates; Nuclear Family; Tyrosinemias | 2000 |
Tissue distribution of 2-(2-nitro-4-trifluoromethylbenzoyl)-cyclohexane-1,3-dione (NTBC) and its effect on enzymes involved in tyrosine catabolism in the mouse.
Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Animals; Aqueous Humor; Cyclohexanones; Dioxygenases; Enzyme Inhibitors; Eye; Homogentisate 1,2-Dioxygenase; Liver; Male; Mice; Mice, Inbred Strains; Nitrobenzoates; Oxygenases; Time Factors; Tissue Distribution; Tyrosine | 2000 |
Cutaneous porphyria in a neonate with tyrosinaemia type 1.
Topics: Combined Modality Therapy; Consanguinity; Coproporphyrins; Cyclohexanones; Enzyme Inhibitors; Humans; Infant, Newborn; Liver Failure; Male; Nitrobenzoates; Photosensitivity Disorders; Phototherapy; Porphyria Cutanea Tarda; Tyrosinemias | 2000 |
[Evolution of a case of tyrosinemia type I treated with NTBC].
Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Adolescent; Carcinoma, Hepatocellular; Cyclohexanones; Enzyme Inhibitors; Humans; Liver Neoplasms; Male; Nitrobenzoates; Palliative Care; Tyrosinemias | 2001 |
Long-term therapy with NTBC and tyrosine-restricted diet in a murine model of hereditary tyrosinemia type I.
Topics: Animals; Cyclohexanones; Diet; Disease Models, Animal; Enzyme Inhibitors; Hydrolases; Mice; Mice, Knockout; Nitrobenzoates; Phenylalanine; Tyrosine; Tyrosinemias | 2002 |
From the Food and Drug Administration.
Topics: Adult; Adverse Drug Reaction Reporting Systems; Anti-Inflammatory Agents, Non-Steroidal; Antidepressive Agents, Second-Generation; Antineoplastic Agents; Arthritis, Psoriatic; Benzamides; Chemical and Drug Induced Liver Injury; Cyclohexanones; Enzyme Inhibitors; Etanercept; Gastrointestinal Neoplasms; Humans; Imatinib Mesylate; Immunoglobulin G; Infant; Liver Failure; Nitrobenzoates; Orphan Drug Production; Piperazines; Pyrimidines; Receptors, Tumor Necrosis Factor; Recombinant Fusion Proteins; Triazoles; Tyrosinemias; United States; United States Food and Drug Administration | 2002 |
Nitisinone. Ntbc, Orfadin.
Topics: Cyclohexanones; Humans; Nitrobenzoates; Tyrosine | 2002 |
Corneal opacities associated with NTBC treatment.
Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Child, Preschool; Cornea; Corneal Opacity; Cyclohexanones; Diet, Protein-Restricted; Enzyme Inhibitors; Female; Humans; Nitrobenzoates; Tyrosine; Tyrosinemias | 2002 |
Tyrosinemia I, a model for human diseases mediated by 2-oxoacid-utilizing dioxygenases: hepatotoxin suppression by NTBC does not normalize hepatic collagen metabolism.
Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Collagen; Cyclohexanones; Enzyme Inhibitors; Humans; Infant; Liver; Models, Molecular; Molecular Structure; Nitrobenzoates; Procollagen-Proline Dioxygenase; Tyrosinemias | 2002 |
Natural history of alkaptonuria.
Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Adolescent; Adult; Age of Onset; Aged; Aged, 80 and over; Alkaptonuria; Child; Child, Preschool; Connective Tissue Diseases; Cyclohexanones; Dioxygenases; Disease Progression; DNA Mutational Analysis; Enzyme Inhibitors; Female; Heart Valve Diseases; Homogentisate 1,2-Dioxygenase; Humans; Joint Diseases; Kidney Calculi; Life Tables; Linear Models; Longitudinal Studies; Male; Middle Aged; Mutation; Nitrobenzoates; Oxygenases; Severity of Illness Index; Tyrosine | 2002 |
Alkaptonuria.
Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Alkaptonuria; Antioxidants; Ascorbic Acid; Benzoquinones; Cyclohexanones; Enzyme Inhibitors; Homogentisic Acid; Humans; Nitrobenzoates; Oxidation-Reduction | 2003 |
Reversibility of cirrhotic regenerative liver nodules upon NTBC treatment in a child with tyrosinaemia type I.
Topics: Cyclohexanones; Enzyme Inhibitors; Humans; Infant; Infant, Newborn; Liver Cirrhosis; Liver Regeneration; Male; Nitrobenzoates; Remission Induction; Tyrosinemias | 2003 |
Pharmacological rescue of the 14CoS/14CoS mouse: hepatocyte apoptosis is likely caused by endogenous oxidative stress.
Topics: Animals; Apoptosis; Caspase 3; Caspases; Cyclohexanones; Free Radicals; Glutamate-Cysteine Ligase; Glutathione; Hepatocytes; Heterozygote; Humans; Hydrolases; Kidney; Liver; Mice; Mice, Knockout; Mice, Transgenic; Microscopy, Electron; Models, Chemical; Nitrobenzoates; Oxidative Stress; Protein Structure, Tertiary; RNA; RNA, Messenger; Time Factors; Tyrosine; Tyrosinemias | 2003 |
Interaction of (4-hydroxyphenyl)pyruvate dioxygenase with the specific inhibitor 2-[2-nitro-4-(trifluoromethyl)benzoyl]-1,3-cyclohexanedione.
Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Cyclohexanones; Electron Spin Resonance Spectroscopy; Enzyme Inhibitors; Enzyme Stability; Ferrous Compounds; Holoenzymes; Kinetics; Magnetic Resonance Spectroscopy; Nitric Oxide; Nitrobenzoates; Oxidation-Reduction; Oxygen; Protein Binding; Spectrophotometry, Ultraviolet; Streptomyces | 2003 |
Extensive changes in liver gene expression induced by hereditary tyrosinemia type I are not normalized by treatment with 2-(2-nitro-4-trifluoromethylbenzoyl)-1,3-cyclohexanedione (NTBC).
Topics: Animals; Blotting, Northern; Cyclohexanones; DNA, Complementary; Enzyme Inhibitors; Female; Gene Expression; Gene Expression Profiling; Liver; Male; Mice; Mice, Inbred C57BL; Mice, Mutant Strains; Nitrobenzoates; Phenotype; Reproducibility of Results; Tyrosinemias | 2003 |
Chronic liver disease in murine hereditary tyrosinemia type 1 induces resistance to cell death.
Topics: Acetaminophen; Analgesics, Non-Narcotic; Animals; Apoptosis; Cell Survival; Central Nervous System Depressants; Chronic Disease; Cyclohexanones; Enzyme Inhibitors; Ethanol; Fas Ligand Protein; fas Receptor; Glutathione; Homogentisic Acid; HSP70 Heat-Shock Proteins; Hydrolases; Liver Failure, Acute; Membrane Glycoproteins; Mice; Mice, Mutant Strains; Necrosis; NF-kappa B; Nitrobenzoates; Oxidative Stress; Proto-Oncogene Proteins c-jun; Tyrosinemias; Up-Regulation | 2004 |
Structure of the ferrous form of (4-hydroxyphenyl)pyruvate dioxygenase from Streptomyces avermitilis in complex with the therapeutic herbicide, NTBC.
Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Amino Acid Sequence; Binding Sites; Crystallography, X-Ray; Cyclohexanones; Enzyme Inhibitors; Enzyme Stability; Herbicides; Iron; Models, Molecular; Molecular Sequence Data; Nitrobenzoates; Protein Conformation; Sequence Homology, Amino Acid; Streptomyces | 2004 |
D-serine-induced nephrotoxicity: possible interaction with tyrosine metabolism.
Topics: Animals; Cyclohexanones; Enzyme Inhibitors; Hydrolases; Kidney Tubules, Proximal; Male; Nitrobenzoates; Phenylpyruvic Acids; Rats; Serine; Tyrosine | 2004 |
Renal proximal tubular cells acquire resistance to cell death stimuli in mice with hereditary tyrosinemia type 1.
Topics: Animals; Caspase 3; Caspase 9; Caspases; Cyclohexanones; Disease Models, Animal; DNA Fragmentation; Enzyme Inhibitors; Hepatocytes; Homogentisic Acid; Hydrolases; In Situ Nick-End Labeling; Kidney Tubules, Proximal; Mice; Mice, Knockout; Nitrobenzoates; Survival Rate; Tyrosine; Tyrosinemias | 2004 |
Experience with NTBC therapy in hereditary tyrosinaemia type I: an alternative to liver transplantation.
Topics: Child, Preschool; Combined Modality Therapy; Cyclohexanones; Enzyme Inhibitors; Female; Follow-Up Studies; Humans; Infant; Liver Transplantation; Male; Nitrobenzoates; Treatment Outcome; Tyrosinemias | 2004 |
Liquid chromatography-tandem mass spectrometry method for the simultaneous determination of delta-ALA, tyrosine and creatinine in biological fluids.
Topics: Aminolevulinic Acid; Calibration; Chromatography, Liquid; Creatinine; Cyclohexanones; Enzyme Inhibitors; Humans; Mass Spectrometry; Nitrobenzoates; Reference Values; Reproducibility of Results; Tyrosine | 2004 |
Successful treatment of severe cardiomyopathy with NTBC in a child with tyrosinaemia type I.
Topics: Amino Acid Metabolism, Inborn Errors; Cardiomyopathy, Hypertrophic; Cyclohexanones; Echocardiography; Enzyme Inhibitors; Humans; Hypertrophy, Left Ventricular; Infant; Male; Nitrobenzoates; Tyrosine; Tyrosinemias | 2005 |
Tandem mass spectrometric assay of succinylacetone in urine for the diagnosis of hepatorenal tyrosinemia.
Topics: Adolescent; Adult; Child; Child, Preschool; Cyclohexanones; Enzyme Inhibitors; Heptanoates; Humans; Infant; Infant, Newborn; Mass Spectrometry; Middle Aged; Neonatal Screening; Nitrobenzoates; Reproducibility of Results; Tyrosinemias | 2005 |
Use of nitisinone in patients with alkaptonuria.
Topics: Adult; Aged; Alkaptonuria; Cyclohexanones; Dietary Proteins; Female; Homogentisic Acid; Humans; Male; Middle Aged; Nitrobenzoates; Tyrosine | 2005 |
Response of metastatic recurrent neuroblastoma to nitisinone: a modulator of tyrosine metabolism.
Topics: Antineoplastic Agents; Antineoplastic Combined Chemotherapy Protocols; Child, Preschool; Combined Modality Therapy; Cyclohexanones; Disease Progression; Dose-Response Relationship, Drug; Drug Administration Schedule; Enzyme Inhibitors; Fatal Outcome; Humans; Male; Neoplasm Recurrence, Local; Neuroblastoma; Nitrobenzoates; Remission Induction; Treatment Outcome; Tyrosine | 2006 |
Involvement of endoplasmic reticulum stress in hereditary tyrosinemia type I.
Topics: Acetoacetates; Animals; Cell Line; Cricetinae; Cyclohexanones; Endoplasmic Reticulum; Endoplasmic Reticulum Chaperone BiP; Enzyme Inhibitors; Eukaryotic Initiation Factor-2; Heat-Shock Proteins; Humans; Hydrolases; Liver; Lung; Mice; Mice, Knockout; Molecular Chaperones; Nitrobenzoates; Proteasome Endopeptidase Complex; Signal Transduction; Transcription, Genetic; Tyrosinemias | 2006 |
Transcriptome analysis of Paracoccidioides brasiliensis cells undergoing mycelium-to-yeast transition.
Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Cell Culture Techniques; Cell Differentiation; Culture Media; Cyclohexanones; Enzyme Inhibitors; Expressed Sequence Tags; Gene Expression Profiling; Gene Expression Regulation, Fungal; Genes, Fungal; Humans; Microarray Analysis; Molecular Structure; Mycelium; Nitrobenzoates; Paracoccidioides; Paracoccidioidomycosis; Temperature; Transcription, Genetic; Yeasts | 2005 |
Kidneys of mice with hereditary tyrosinemia type I are extremely sensitive to cytotoxicity.
Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Animals; Cyclohexanones; Enzyme Inhibitors; Female; Homogentisic Acid; Humans; Hydrolases; In Situ Nick-End Labeling; Kidney; Liver; Male; Mice; Mice, Inbred C57BL; Mice, Knockout; Nitrobenzoates; Tyrosinemias | 2006 |
Tyrosinemia produced by 2-(2-nitro-4-trifluoromethylbenzoyl)-cyclohexane-1,3-dione (NTBC) in experimental animals and its relationship to corneal injury.
Topics: Animals; Cornea; Corneal Injuries; Cyclohexanones; Dogs; Dose-Response Relationship, Drug; Homogentisate 1,2-Dioxygenase; Macaca mulatta; Male; Nitrobenzoates; Rabbits; Tyrosine Transaminase; Tyrosinemias | 2006 |
Evaluation of dichloroacetate treatment in a murine model of hereditary tyrosinemia type 1.
Topics: Animals; Cyclohexanones; Dichloroacetic Acid; Female; Glutathione; Glutathione Disulfide; Hydrolases; Kidney; Liver; Male; Mice; Nitrobenzoates; Oxidative Stress; Tyrosine; Tyrosinemias; Weight Loss | 2006 |
Cardiomyopathy in tyrosinaemia type I is common but usually benign.
Topics: Adolescent; Cardiomyopathies; Child; Child, Preschool; Cyclohexanones; Echocardiography; Enzyme Inhibitors; Female; Humans; Infant; Infant, Newborn; Male; Nitrobenzoates; Retrospective Studies; Treatment Outcome; Tyrosinemias | 2006 |
Nitisinone in the treatment of hereditary tyrosinaemia type 1.
Topics: Carcinoma, Hepatocellular; Cyclohexanones; Enzyme Inhibitors; Humans; Kidney Diseases; Liver Diseases; Liver Failure; Liver Neoplasms; Liver Transplantation; Monitoring, Physiologic; Nitrobenzoates; Tyrosine; Tyrosinemias | 2006 |
Tyrosinemia type I treated by NTBC: how does AFP predict liver cancer?
Topics: alpha-Fetoproteins; Child; Child, Preschool; Cyclohexanones; Enzyme Inhibitors; Female; Humans; Infant; Liver Neoplasms; Male; Nitrobenzoates; Prognosis; Tyrosinemias | 2006 |
[New drugs; nitisinone].
Topics: Cyclohexanones; Enzyme Inhibitors; Humans; Nitrobenzoates; Tyrosine; Tyrosinemias | 2006 |
Rescue from neonatal death in the murine model of hereditary tyrosinemia by glutathione monoethylester and vitamin C treatment.
Topics: Animals; Animals, Newborn; Antioxidants; Ascorbic Acid; Carcinoma, Hepatocellular; Cyclohexanones; Disease Models, Animal; Drug Therapy, Combination; Female; Glutathione; Hydrolases; Liver Neoplasms; Mice; Mice, Knockout; Nitrobenzoates; Oxidative Stress; Pregnancy; Tyrosinemias | 2008 |
Hepatic stress in hereditary tyrosinemia type 1 (HT1) activates the AKT survival pathway in the fah-/- knockout mice model.
Topics: Animals; bcl-X Protein; Carcinoma, Hepatocellular; Cyclohexanones; Disease Models, Animal; Hydrolases; Liver Neoplasms; Mice; Mice, Knockout; Nitrobenzoates; Proto-Oncogene Proteins c-akt; Proto-Oncogene Proteins c-bcl-2; Signal Transduction; Tyrosinemias | 2008 |
NTBC treatment in tyrosinaemia type I: long-term outcome in French patients.
Topics: Child, Preschool; Cyclohexanones; Enzyme Inhibitors; Follow-Up Studies; France; Humans; Infant; Infant, Newborn; Liver; Liver Function Tests; Nitrobenzoates; Patient Compliance; Retrospective Studies; Time Factors; Treatment Failure; Tyrosinemias | 2008 |
Severe neurological crisis in a patient with hereditary tyrosinaemia type I after interruption of NTBC treatment.
Topics: Cyclohexanones; Drug Administration Schedule; Enzyme Inhibitors; Humans; Hypertension; Infant; Male; Medication Adherence; Nitrobenzoates; Polyneuropathies; Respiration, Artificial; Respiratory Insufficiency; Respiratory Paralysis; Tyrosinemias | 2008 |
Renal tubular function in children with tyrosinaemia type I treated with nitisinone.
Topics: Child; Child, Preschool; Cyclohexanones; Female; Humans; Infant; Infant, Newborn; Kidney; Kidney Tubules; Male; Nitrobenzoates; Proteinuria; Retrospective Studies; Tyrosinemias; Ultrasonography | 2008 |
Loss of p21 permits carcinogenesis from chronically damaged liver and kidney epithelial cells despite unchecked apoptosis.
Topics: Animals; Apoptosis; Carcinoma, Hepatocellular; Cell Cycle; Cell Proliferation; Cyclin-Dependent Kinase Inhibitor p21; Cyclohexanones; Disease Models, Animal; Enzyme Inhibitors; Hepatectomy; Hepatocytes; Hydrolases; Kidney Diseases, Cystic; Kidney Neoplasms; Kidney Tubules, Proximal; Liver Neoplasms; Liver Regeneration; Mice; Mice, Knockout; Neoplasms; Nitrobenzoates; Tyrosinemias | 2008 |
Activation of nuclear factor E2-related factor 2 in hereditary tyrosinemia type 1 and its role in survival and tumor development.
Topics: Animals; Cyclohexanones; DNA Damage; Dose-Response Relationship, Drug; Glutathione; Hepatitis; Hydrolases; Imidazoles; Inactivation, Metabolic; Liver; Liver Cirrhosis; Liver Failure; Liver Neoplasms; Mice; Mice, Knockout; NF-E2-Related Factor 2; Nitrobenzoates; Oleanolic Acid; Oxidative Stress; Tyrosinemias | 2008 |
Identification of 2-[2-nitro-4-(trifluoromethyl)benzoyl]- cyclohexane-1,3-dione metabolites in urine of patients suffering from tyrosinemia type I with the use of 1H and 19F NMR spectroscopy.
Topics: Child; Child, Preschool; Cyclohexanones; Female; Fluorine; Humans; Magnetic Resonance Spectroscopy; Male; Nitrobenzoates; Protons; Tyrosinemias | 2008 |
Liquid chromatography tandem mass spectrometry method for the quantitation of NTBC (2-(nitro-4-trifluoromethylbenzoyl)1,3-cyclohexanedione) in plasma of tyrosinemia type 1 patients.
Topics: Chromatography, Liquid; Cyclohexanones; Humans; Nitrobenzoates; Tandem Mass Spectrometry; Tyrosinemias | 2009 |
Clinical practice. NTBC therapy for tyrosinemia type 1: how much is enough?
Topics: Child, Preschool; Cost-Benefit Analysis; Cyclohexanones; Dose-Response Relationship, Drug; Drug Costs; Egypt; Enzyme Inhibitors; Female; Heptanoates; Humans; Infant; Nitrobenzoates; Tyrosinemias | 2010 |
Determination of NTBC in serum samples from patients with hereditary tyrosinemia type I by capillary electrophoresis.
Topics: Cyclohexanones; Electrophoresis, Capillary; Enzyme Inhibitors; Humans; Limit of Detection; Nitrobenzoates; Time Factors; Tyrosinemias | 2010 |
Identification of NTBC metabolites in urine from patients with hereditary tyrosinemia type 1 using two different mass spectrometric platforms: triple stage quadrupole and LTQ-Orbitrap.
Topics: Cyclohexanones; Humans; Mass Spectrometry; Models, Molecular; Molecular Weight; Nitrobenzoates; Toluene; Tyrosinemias | 2010 |
High volume naked DNA tail-vein injection restores liver function in Fah-knock out mice.
Topics: Animals; Bilirubin; Biomarkers; Cell Proliferation; Cyclohexanones; Dependovirus; Disease Models, Animal; Genetic Therapy; Genetic Vectors; Hepatocytes; Hydrolases; Injections, Intravenous; L-Lactate Dehydrogenase; Liver; Liver Function Tests; Mice; Mice, Inbred BALB C; Mice, Knockout; Nitrobenzoates; Plasmids; Serum Albumin; Tail; Time Factors; Transfection; Tyrosinemias; Urea | 2010 |
Tyrosinemia type 1: metastatic hepatoblastoma with a favorable outcome.
Topics: Adolescent; Biopsy, Needle; Carcinoma, Hepatocellular; Chemotherapy, Adjuvant; Cyclohexanones; Diagnosis, Differential; Female; Follow-Up Studies; Hepatectomy; Hepatoblastoma; Humans; Immunohistochemistry; Liver Neoplasms; Nitrobenzoates; Risk Assessment; Treatment Outcome; Tyrosinemias | 2010 |
Product analysis and inhibition studies of a causative Asn to Ser variant of 4-hydroxyphenylpyruvate dioxygenase suggest a simple route to the treatment of Hawkinsinuria.
Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Amino Acid Sequence; Animals; Cyclohexanones; Escherichia coli; Gene Expression; Humans; Infant; Metabolic Diseases; Models, Molecular; Molecular Sequence Data; Mutation; Nitrobenzoates; Quinones; Rats; Sequence Alignment; Streptomyces | 2010 |
Monitoring tyrosinaemia type I: Blood spot test for nitisinone (NTBC).
Topics: Blood Chemical Analysis; Cyclohexanones; Follow-Up Studies; Humans; Male; Nitrobenzoates; Specimen Handling; Treatment Outcome; Tyrosinemias | 2011 |
Increase of CSF tyrosine and impaired serotonin turnover in tyrosinemia type I.
Topics: Brain; Child; Cyclohexanones; Enzyme Inhibitors; Humans; Infant; Male; Nitrobenzoates; Psychomotor Performance; Serotonin; Tyrosine; Tyrosinemias | 2011 |
Alkaptonuria.
Topics: Aged; Alkaptonuria; Arthritis; Cyclohexanones; Female; Homogentisic Acid; Humans; Joint Diseases; Nitrobenzoates; Ochronosis; Pigmentation Disorders | 2010 |
Comparison of plasma and dry blood spots as samples for the determination of nitisinone (NTBC) by high-performance liquid chromatography-tandem mass spectrometry. Study of the stability of the samples at different temperatures.
Topics: Adolescent; Analysis of Variance; Blood Specimen Collection; Child; Child, Preschool; Chromatography, High Pressure Liquid; Cyclohexanones; Drug Stability; Female; Humans; Male; Nitrobenzoates; Reproducibility of Results; Sensitivity and Specificity; Tandem Mass Spectrometry; Temperature; Time Factors; Tyrosinemias | 2011 |
Hereditary tyrosinemia type 1 from a single center in Egypt: clinical study of 22 cases.
Topics: Child, Preschool; Cyclohexanones; Egypt; Enzyme Inhibitors; Female; Humans; Infant; Liver Transplantation; Male; Nitrobenzoates; Tyrosinemias | 2011 |
A quantitative assessment of alkaptonuria: testing the reliability of two disease severity scoring systems.
Topics: Adolescent; Adult; Age Distribution; Aged; Aged, 80 and over; Alkaptonuria; Causality; Cohort Studies; Comorbidity; Cyclohexanones; Female; Homogentisate 1,2-Dioxygenase; Homogentisic Acid; Humans; Hypertension; Male; Middle Aged; Nitrobenzoates; Ochronosis; Pain; Regression Analysis; Reproducibility of Results; Severity of Illness Index; Sex Distribution; Smoking; Surveys and Questionnaires | 2011 |
Natural history of alkaptonuria revisited: analyses based on scoring systems.
Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Adolescent; Adult; Aged; Aged, 80 and over; Alkaptonuria; Arthralgia; Arthritis; Cartilage, Articular; Causality; Cohort Studies; Comorbidity; Cyclohexanones; Disease Progression; Female; Homogentisate 1,2-Dioxygenase; Homogentisic Acid; Humans; Male; Middle Aged; Nitrobenzoates; Ochronosis; Pain; Surveys and Questionnaires | 2011 |
Tyrosinemia type 1 in Spain: mutational analysis, treatment and long-term outcome.
Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Child; Child, Preschool; Cyclohexanones; DNA; DNA Mutational Analysis; Enzyme Inhibitors; Female; Follow-Up Studies; Genotype; Humans; Incidence; Male; Mutation; Nitrobenzoates; Retrospective Studies; Spain; Time Factors; Treatment Outcome; Tyrosine; Tyrosinemias | 2011 |
Generation of healthy mice from gene-corrected disease-specific induced pluripotent stem cells.
Topics: Animals; Cell Survival; Cells, Cultured; Chromosomes; Cyclohexanones; Disease Models, Animal; Female; Fetus; Fibroblasts; Genetic Complementation Test; Genetic Therapy; Genetic Vectors; Humans; Hydrolases; Induced Pluripotent Stem Cells; Lentivirus; Liver; Mice; Mice, Knockout; Nitrobenzoates; Pregnancy; Promoter Regions, Genetic; Spleen Focus-Forming Viruses; Tetraploidy; Tyrosinemias | 2011 |
Alkaptonuria: treasure your exceptions.
Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Alkaptonuria; Cyclohexanones; Humans; Nitrobenzoates; Therapies, Investigational | 2011 |
Informed reasoning: repositioning of nitisinone to treat oculocutaneous albinism.
Topics: Albinism, Oculocutaneous; Animals; Cyclohexanones; Eye Color; Female; Humans; Nitrobenzoates; Pregnancy; Skin Pigmentation | 2011 |
Nitisinone improves eye and skin pigmentation defects in a mouse model of oculocutaneous albinism.
Topics: Albinism, Oculocutaneous; Animals; Cyclohexanones; Disease Models, Animal; Enzyme Inhibitors; Enzyme Stability; Eye Color; Female; Humans; Melanins; Melanocytes; Mice; Mice, Inbred C57BL; Mice, Mutant Strains; Microscopy, Electron, Transmission; Models, Molecular; Monophenol Monooxygenase; Mutation; Nitrobenzoates; Pregnancy; Skin Pigmentation; Tyrosine | 2011 |
Neurocognitive outcome in patients with hypertyrosinemia type I after long-term treatment with NTBC.
Topics: Cerebrum; Child; Child, Preschool; Cognition; Cognition Disorders; Cyclohexanones; Enzyme Inhibitors; Female; Humans; Infant; Language Development; Long-Term Care; Male; Motor Activity; Nitrobenzoates; Psychometrics; Time; Treatment Outcome; Tyrosine; Tyrosinemias | 2012 |
Grown up mice from gene-corrected iPS cells.
Topics: Animals; Cell Differentiation; Cyclohexanones; Embryo Transfer; Gene Silencing; Genetic Complementation Test; Genetic Vectors; Hydrolases; Induced Pluripotent Stem Cells; Lentivirus; Liver; Mice; Mice, Knockout; Mice, Transgenic; Nitrobenzoates; Transduction, Genetic; Transgenes; Tyrosinemias | 2011 |
LC-MS/MS method for simultaneous determination on a dried blood spot of multiple analytes relevant for treatment monitoring in patients with tyrosinemia type I.
Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Adolescent; Biomarkers; Child; Child, Preschool; Chromatography, Liquid; Cyclohexanones; Diet, Protein-Restricted; Drug Monitoring; Enzyme Inhibitors; Heptanoates; Humans; Infant; Infant, Newborn; Kidney; Liver; Methionine; Nitrobenzoates; Phenylalanine; Tandem Mass Spectrometry; Tyrosine; Tyrosinemias | 2012 |
Single dose NTBC-treatment of hereditary tyrosinemia type I.
Topics: Cyclohexanones; Drug Administration Schedule; Female; Heptanoates; Humans; Infant; Infant, Newborn; Male; Nitrobenzoates; Tyrosinemias | 2012 |
Plasma succinylacetone is persistently raised after liver transplantation in tyrosinaemia type 1.
Topics: Adolescent; Child; Child, Preschool; Cyclohexanones; Heptanoates; Humans; Infant; Liver Transplantation; Nitrobenzoates; Porphobilinogen Synthase; Retrospective Studies; Tyrosinemias | 2013 |
Confocal microscopy of corneal crystals in a patient with hereditary tyrosinemia type I, treated with NTBC.
Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Adolescent; Corneal Opacity; Crystallization; Cyclohexanones; Enzyme Inhibitors; Epithelium, Corneal; Fluorescein; Humans; Male; Microscopy, Confocal; Nitrobenzoates; Staining and Labeling; Tyrosinemias | 2013 |
Simple and fast quantification of nitisone (NTBC) using liquid chromatography-tandem mass spectrometry method in plasma of tyrosinemia type 1 patients.
Topics: Adolescent; Child; Child, Preschool; Chromatography, High Pressure Liquid; Cyclohexanones; Enzyme Inhibitors; Humans; Infant; Nitrobenzoates; Sensitivity and Specificity; Spectrometry, Mass, Electrospray Ionization; Tandem Mass Spectrometry; Tyrosinemias | 2012 |
Metabolic liver disease.
Topics: 1-Deoxynojirimycin; Cyclohexanones; Diet; Enzyme Inhibitors; Enzyme Replacement Therapy; Hepatocytes; Hepatomegaly; Humans; Hydrops Fetalis; Infant; Infant, Newborn; Jaundice, Neonatal; Liver Diseases; Liver Transplantation; Metabolism, Inborn Errors; Nitrobenzoates; Splenomegaly | 2012 |
[Clinical follow up of Chilean patients with tyrosinemia type 1 treated with 2-(2-nitro-4-trifluoromethylbenzoyl)-1,3-ciclohexanedione (NTBC)].
Topics: Child, Preschool; Chile; Cyclohexanones; Enzyme Inhibitors; Female; Follow-Up Studies; Humans; Infant; Liver Neoplasms; Liver Transplantation; Male; Nitrobenzoates; Retrospective Studies; Time Factors; Treatment Outcome; Tyrosinemias | 2012 |
Effect of nitisinone (NTBC) treatment on the clinical course of hepatorenal tyrosinemia in Québec.
Topics: Child; Child, Preschool; Cyclohexanones; Enzyme Inhibitors; Humans; Infant; Infant, Newborn; Kidney; Liver; Liver Transplantation; Neonatal Screening; Nitrobenzoates; Quebec; Treatment Outcome; Tyrosinemias | 2012 |
Corneal depositions in tyrosinaemia type I during treatment with Nitisinone.
Topics: Adolescent; Corneal Diseases; Cyclohexanones; Enzyme Inhibitors; Eye Pain; Humans; Male; Nitrobenzoates; Patient Compliance; Photophobia; Tyrosinemias | 2012 |
Maternal and fetal tyrosinemia type I.
Topics: Biomarkers; Child Development; Consanguinity; Cyclohexanones; Diet, Protein-Restricted; DNA Mutational Analysis; Female; Genetic Predisposition to Disease; Heptanoates; Heredity; Heterozygote; Homozygote; Humans; Hydrolases; Infant; Infant, Newborn; Live Birth; Mutation; Nitrobenzoates; Pedigree; Phenotype; Pregnancy; Tyrosine; Tyrosinemias; Young Adult | 2010 |
Ochronotic osteoarthropathy in a mouse model of alkaptonuria, and its inhibition by nitisinone.
Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Alkaptonuria; Animals; Chondrocytes; Cyclohexanones; Disease Models, Animal; Enzyme Inhibitors; Female; Humans; Joint Diseases; Knee Joint; Male; Mice; Mice, Inbred BALB C; Mice, Knockout; Nitrobenzoates; Ochronosis | 2014 |
Pregnancy in an NTBC-treated patient with hereditary tyrosinemia type I.
Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Adolescent; Combined Modality Therapy; Contraindications; Cyclohexanones; Enzyme Inhibitors; Female; Humans; Nitrobenzoates; Phenylalanine; Pregnancy; Pregnancy Complications; Term Birth; Tyrosine; Tyrosinemias | 2015 |
Tyrosinemia type 1: a rare and forgotten cause of reversible hypertrophic cardiomyopathy in infancy.
Topics: Cardiomyopathy, Hypertrophic; Cyclohexanones; Food, Formulated; Heptanoates; Homozygote; Humans; Hydrolases; Infant; Male; Mutation; Nitrobenzoates; Siblings; Treatment Outcome; Tyrosinemias | 2013 |
Impaired cognitive functioning in patients with tyrosinemia type I receiving nitisinone.
Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Adolescent; Child; Child Development; Child, Preschool; Cognition; Cognitive Dysfunction; Cross-Sectional Studies; Cyclohexanones; Disease Progression; Enzyme Inhibitors; Female; Humans; Incidence; Male; Netherlands; Nitrobenzoates; Prognosis; Risk Factors; Tyrosinemias; Young Adult | 2014 |
Pharmacologic inhibition of L-tyrosine degradation ameliorates cerebral dopamine deficiency in murine phenylketonuria (PKU).
Topics: Amino Acids; Animals; Brain Chemistry; Cyclohexanones; Dopamine; Enzyme Inhibitors; Mice; Mice, Inbred C57BL; Mice, Knockout; Neurotransmitter Agents; Nitrobenzoates; Phenylketonurias; Tyrosine | 2014 |
Early nitisinone treatment reduces the need for liver transplantation in children with tyrosinaemia type 1 and improves post-transplant renal function.
Topics: Adolescent; Adult; Child; Child, Preschool; Cyclohexanones; Enzyme Inhibitors; Female; Glomerular Filtration Rate; Humans; Hypertension; Kidney; Liver Transplantation; Male; Nitrobenzoates; Retrospective Studies; Treatment Outcome; Tyrosinemias; Young Adult | 2014 |
The fate of tyrosinaemic Hungarian patients before the NTBC aera.
Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Carcinoma, Hepatocellular; Carcinoma, Renal Cell; Child, Preschool; Cyclohexanones; Enzyme Inhibitors; Fatal Outcome; Homozygote; Humans; Hungary; Hydrolases; Kidney Neoplasms; Liver Neoplasms; Male; Nitrobenzoates; Treatment Outcome; Tyrosine; Tyrosinemias | 2013 |
NTBC treatment of the pyomelanogenic Pseudomonas aeruginosa clinical isolate PA1111 inhibits pigment production and increases sensitivity to oxidative stress.
Topics: Cyclohexanones; Melanins; Nitrobenzoates; Oxidative Stress; Pseudomonas aeruginosa | 2014 |
Infiltrating neutrophils aggravate metabolic liver failure in fah-deficient mice.
Topics: Animals; Bilirubin; Body Weight; Cyclohexanones; Disease Models, Animal; Hepatocytes; Hydrolases; Inflammation; Interleukin-22; Interleukins; Liver; Liver Failure; Male; Mice; Mice, Knockout; Neutrophils; Nitrobenzoates; Receptors, CCR2; Tyrosine | 2015 |
HCC prevalence and histopathological findings in liver explants of patients with hereditary tyrosinemia type 1.
Topics: Carcinoma, Hepatocellular; Child; Child, Preschool; Cyclohexanones; Drug Resistance; Enzyme Inhibitors; Female; Follow-Up Studies; Humans; Infant; Liver; Liver Neoplasms; Liver Transplantation; Male; Nitrobenzoates; Prognosis; Retrospective Studies; Tomography, X-Ray Computed; Tyrosinemias | 2014 |
Cross-sectional study of 168 patients with hepatorenal tyrosinaemia and implications for clinical practice.
Topics: Adolescent; Child; Child, Preschool; Cross-Sectional Studies; Cyclohexanones; Enzyme Inhibitors; Female; Follow-Up Studies; Humans; Infant; Infant, Newborn; Liver Failure; Liver Transplantation; Male; Neonatal Screening; Nitrobenzoates; Rare Diseases; Renal Insufficiency; Retrospective Studies; Surveys and Questionnaires; Treatment Outcome; Tyrosinemias; Young Adult | 2014 |
Early effect of NTBC on renal tubular dysfunction in hereditary tyrosinemia type 1.
Topics: Biomarkers; Child, Preschool; Cyclohexanones; Female; Humans; Infant; Kidney Tubules; Male; Nitrobenzoates; Retrospective Studies; Treatment Outcome; Tyrosinemias | 2014 |
Hereditary tyrosinemia type 1 in Turkey: twenty year single-center experience.
Topics: Acute Disease; Child; Child, Preschool; Chronic Disease; Cyclohexanones; Diet Therapy; Early Diagnosis; Enzyme Inhibitors; Female; Hepatomegaly; Humans; Infant; Infant, Newborn; Kidney Diseases; Liver Transplantation; Living Donors; Male; Nitrobenzoates; Prognosis; Retrospective Studies; Splenomegaly; Turkey; Tyrosinemias | 2015 |
Outcome of children with hereditary tyrosinaemia following newborn screening.
Topics: alpha-Fetoproteins; Cyclohexanones; Enzyme Inhibitors; Female; Genotype; Humans; Hydrolases; Infant, Newborn; Male; Mutation; Neonatal Screening; Nitrobenzoates; Prothrombin Time; Treatment Outcome; Tyrosinemias | 2015 |
Serum markers in alkaptonuria: simultaneous analysis of homogentisic acid, tyrosine and nitisinone by liquid chromatography tandem mass spectrometry.
Topics: Alkaptonuria; Biomarkers; Chromatography, Liquid; Cyclohexanones; Homogentisic Acid; Humans; Nitrobenzoates; Tandem Mass Spectrometry; Tyrosine | 2015 |
Perturbations of tyrosine metabolism promote the indolepyruvate pathway via tryptophan in host and microbiome.
Topics: Aldehydes; Alkaptonuria; Cell Line, Tumor; Cyclohexanones; Gastrointestinal Microbiome; Humans; Indoles; Mass Spectrometry; Metabolomics; Nitrobenzoates; Phenylpyruvic Acids; Symbiosis; Tryptophan; Tyrosine; Tyrosinemias | 2015 |
Neonatal screening for hereditary tyrosinaemia: are we there yet?
Topics: Cyclohexanones; Enzyme Inhibitors; Female; Humans; Male; Neonatal Screening; Nitrobenzoates; Tyrosinemias | 2015 |
Osteoarticular cells tolerate short-term exposure to nitisinone-implications in alkaptonuria.
Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Alkaptonuria; Cell Line, Tumor; Chondrocytes; Cyclohexanones; Drug Evaluation, Preclinical; Humans; Nitrobenzoates; Osteoblasts | 2016 |
Progress in Alkaptonuria--are we near to an effective therapy?
Topics: Alkaptonuria; Animals; Cyclohexanones; Disease Models, Animal; Genetic Therapy; Humans; Mice; Nitrobenzoates; Ochronosis; Treatment Outcome | 2015 |
Natural Killer Cells-Produced IFN-γ Improves Bone Marrow-Derived Hepatocytes Regeneration in Murine Liver Failure Model.
Topics: Animals; Bone Marrow Cells; Bone Marrow Transplantation; Cell Fusion; Cyclohexanones; Disease Models, Animal; Female; Hepatocytes; Hydrolases; Interferon gamma Receptor; Interferon-gamma; Killer Cells, Natural; Liver Failure; Liver Regeneration; Male; Mice; Mice, Knockout; Monocytes; Nitrobenzoates; Protein Binding; Receptors, Interferon | 2015 |
Methods to Inhibit Bacterial Pyomelanin Production and Determine the Corresponding Increase in Sensitivity to Oxidative Stress.
Topics: Anti-Bacterial Agents; Cyclohexanones; Hydrogen Peroxide; Melanins; Microbial Sensitivity Tests; Nitrobenzoates; Oxidative Stress; Pseudomonas aeruginosa; Reproducibility of Results | 2015 |
[Pharmacological and clinical profile of nitisinone (Orfadin(®) Capsules): a therapeutic agent for hereditary tyrosinemia type 1].
Topics: Animals; Capsules; Clinical Trials as Topic; Cyclohexanones; Enzyme Inhibitors; Humans; Nitrobenzoates; Signal Transduction; Tyrosinemias | 2015 |
Inhibition of para-Hydroxyphenylpyruvate Dioxygenase by Analogues of the Herbicide Nitisinone As a Strategy to Decrease Homogentisic Acid Levels, the Causative Agent of Alkaptonuria.
Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Alkaptonuria; Animals; Cell Survival; Cyclohexanones; Dose-Response Relationship, Drug; Herbicides; Homogentisic Acid; Humans; Male; Molecular Structure; Nitrobenzoates; Rats; Rats, Wistar; Structure-Activity Relationship | 2016 |
Tyrosinemia type I: Case series with response to treatment to NTBC.
Topics: Child, Preschool; Cyclohexanones; Female; Humans; Infant; Male; Nitrobenzoates; Treatment Outcome; Tyrosinemias | 2016 |
Tyrosinemia type 1 and irreversible neurologic crisis after one month discontinuation of nitisone.
Topics: Cyclohexanones; Fatal Outcome; Humans; Infant; Male; Nervous System Diseases; Nitrobenzoates; Time Factors; Tyrosine; Tyrosinemias | 2016 |
Tyrosinemia type I and not treatment with NTBC causes slower learning and altered behavior in mice.
Topics: Animals; Behavior, Animal; Cyclohexanones; Disease Models, Animal; Hydrolases; Learning; Memory, Long-Term; Mice; Nitrobenzoates; Tyrosine; Tyrosinemias | 2016 |
Predicting tyrosinaemia: a mathematical model of 4-hydroxyphenylpyruvate dioxygenase inhibition by nitisinone in rats.
Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Animals; Computer Simulation; Cyclohexanones; Enzyme Inhibitors; Kinetics; Liver; Mathematical Concepts; Models, Animal; Models, Biological; Nitrobenzoates; Rats; Tyrosine; Tyrosinemias | 2017 |
Curative ex vivo liver-directed gene therapy in a pig model of hereditary tyrosinemia type 1.
Topics: Animals; Cyclohexanones; Disease Models, Animal; Genetic Therapy; Hepatocytes; Hydrolases; Immunohistochemistry; Liver; Nitrobenzoates; Swine; Transplantation, Homologous; Tyrosinemias | 2016 |
Reprogramming metabolic pathways in vivo with CRISPR/Cas9 genome editing to treat hereditary tyrosinaemia.
Topics: Animals; Cell Line; Cell Proliferation; CRISPR-Cas Systems; Cyclohexanones; Disease Models, Animal; Enzyme Inhibitors; Exons; Gene Editing; Genetic Therapy; Hepatocytes; Humans; Hydrolases; Liver; Metabolic Networks and Pathways; Mice; Mice, Inbred Strains; Mice, Knockout; Nitrobenzoates; Oxidoreductases; Phenotype; Tyrosinemias | 2016 |
A 54-year-old woman with arthritis and discoloration of the hands, ears, and sclerae.
Topics: Alkaptonuria; Arthroplasty, Replacement, Knee; Cyclohexanones; Diagnosis, Differential; Ear; Female; Hand; Humans; Middle Aged; Nitrobenzoates; Palliative Care; Pigmentation Disorders; Prognosis; Prosthesis Failure; Reoperation; Sclera; Severity of Illness Index | 2017 |
The outcome of seven patients with hereditary tyrosinemia type 1.
Topics: Biomarkers; Child; Child, Preschool; Cyclohexanones; Echocardiography; Enzyme Inhibitors; Female; Gas Chromatography-Mass Spectrometry; Humans; Infant; Magnetic Resonance Imaging; Male; Nitrobenzoates; Retrospective Studies; Treatment Outcome; Tyrosinemias; Ultrasonography | 2016 |
In brief: Nitisinone (Orfadin) for hereditary tyrosinemia.
Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Administration, Oral; Capsules; Cyclohexanones; Drug Administration Schedule; Enzyme Inhibitors; Humans; Nitrobenzoates; Pharmaceutical Solutions; Treatment Outcome; Tyrosinemias | 2016 |
Deranged Liver Function in a Neonate.
Topics: Biomarkers; Cyclohexanones; Enzyme Inhibitors; Female; Gas Chromatography-Mass Spectrometry; Heptanoates; Humans; Infant, Newborn; Liver Diseases; Neonatal Screening; Nitrobenzoates; Tyrosinemias; Urinalysis | 2016 |
Type 1 Tyrosinaemia.
Topics: Biomarkers; Cyclohexanones; Enzyme Inhibitors; Heptanoates; Humans; Hydrolases; Hypoglycemia; Ireland; Liver Transplantation; Mutation; Nitrobenzoates; Tyrosinemias | 2016 |
The effect of nitisinone on homogentisic acid and tyrosine: a two-year survey of patients attending the National Alkaptonuria Centre, Liverpool.
Topics: Adult; Aged; Alkaptonuria; Chromatography, Liquid; Cyclohexanones; Drug Administration Schedule; Enzyme Inhibitors; Female; Homogentisate 1,2-Dioxygenase; Homogentisic Acid; Humans; Longitudinal Studies; Male; Middle Aged; Monitoring, Physiologic; Nitrobenzoates; Tandem Mass Spectrometry; Tyrosine; United Kingdom | 2017 |
Tyrosinemia Type I in Japan: A Report of Five Cases.
Topics: Child, Preschool; Cyclohexanones; Female; Humans; Infant; Japan; Liver; Liver Transplantation; Male; Nitrobenzoates; Phenylalanine; Tyrosine; Tyrosinemias | 2017 |
Daily variation of NTBC and its relation to succinylacetone in tyrosinemia type 1 patients comparing a single dose to two doses a day.
Topics: Adolescent; Child; Child, Preschool; Chromatography, High Pressure Liquid; Cyclohexanones; Diet, Protein-Restricted; Dried Blood Spot Testing; Drug Administration Schedule; Drug Monitoring; Female; Humans; Infant; Male; Nitrobenzoates; Prospective Studies; Tandem Mass Spectrometry; Time Factors; Treatment Outcome; Tyrosinemias; Young Adult | 2018 |
Simultaneous quantification of succinylacetone and nitisinone for therapeutic drug monitoring in the treatment of Tyrosinemia type 1.
Topics: Chromatography, Liquid; Cyclohexanones; Drug Monitoring; Heptanoates; Humans; Linear Models; Nitrobenzoates; Reproducibility of Results; Sensitivity and Specificity; Tandem Mass Spectrometry; Tyrosinemias | 2018 |
Clinical and biochemical assessment of depressive symptoms in patients with Alkaptonuria before and after two years of treatment with nitisinone.
Topics: Adolescent; Adult; Aged; Alkaptonuria; Cyclohexanones; Depression; Dopamine; Female; Humans; Hydroxyindoleacetic Acid; Male; Middle Aged; Nitrobenzoates; Tyrosine; Young Adult | 2018 |
Nitisinone arrests ochronosis and decreases rate of progression of Alkaptonuria: Evaluation of the effect of nitisinone in the United Kingdom National Alkaptonuria Centre.
Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Alkaptonuria; Cyclohexanones; Disease Progression; Female; Homogentisic Acid; Humans; Male; Middle Aged; Nitrobenzoates; Ochronosis; United Kingdom | 2018 |
Minimal Efficacy of Nitisinone Treatment in a Novel Mouse Model of Oculocutaneous Albinism, Type 3.
Topics: Administration, Oral; Albinism, Oculocutaneous; Animals; Blotting, Western; Cyclohexanones; Disease Models, Animal; Enzyme Inhibitors; Genotyping Techniques; Melanins; Melanosomes; Membrane Glycoproteins; Mice; Mice, Inbred BALB C; Mice, Inbred C57BL; Mice, Inbred DBA; Microscopy, Electron, Transmission; Nitrobenzoates; Oxidoreductases; Real-Time Polymerase Chain Reaction; Treatment Outcome; Tyrosine | 2018 |
Discontinuation of NTBC after liver transplantation in tyrosinemia type 1.
Topics: Cyclohexanones; Enzyme Inhibitors; Humans; Infant; Liver Transplantation; Magnetic Resonance Imaging; Male; Nitrobenzoates; Tomography, X-Ray Computed; Tyrosinemias; Withholding Treatment | 2018 |
A father's fight to help his sons - and fix clinical trials.
Topics: Adolescent; Alkaptonuria; Animals; Autopsy; Child; Clinical Trials as Topic; Compassionate Use Trials; Cyclohexanones; Disease Models, Animal; Disease Progression; Drug Approval; Endpoint Determination; Female; Homogentisic Acid; Humans; Infant; Male; Mice; National Health Programs; National Institutes of Health (U.S.); Nitrobenzoates; Observational Studies as Topic; Off-Label Use; Orphan Drug Production; Rare Diseases; Rats; Sample Size; Tyrosine; Tyrosinemias; United States; United States Food and Drug Administration | 2019 |
Molecular insights into the mechanism of 4-hydroxyphenylpyruvate dioxygenase inhibition: enzyme kinetics, X-ray crystallography and computational simulations.
Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Amino Acid Sequence; Computer Simulation; Crystallography, X-Ray; Cyclohexanones; Enzyme Inhibitors; Herbicides; Kinetics; Mesylates; Molecular Structure; Nitrobenzoates; Sequence Homology, Amino Acid | 2019 |
Hydrophobicity-oriented drug design (HODD) of new human 4-hydroxyphenylpyruvate dioxygenase inhibitors.
Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Catalytic Domain; Cyclohexanones; Drug Design; Enzyme Inhibitors; Humans; Hydrophobic and Hydrophilic Interactions; Molecular Docking Simulation; Nitrobenzoates; Structure-Activity Relationship | 2019 |
A Comprehensive LC-QTOF-MS Metabolic Phenotyping Strategy: Application to Alkaptonuria.
Topics: Aged; Alkaptonuria; Animals; Chromatography, Liquid; Cyclohexanones; Databases, Chemical; Female; Gene Knockdown Techniques; Homogentisate 1,2-Dioxygenase; Humans; Male; Mass Spectrometry; Metabolome; Metabolomics; Mice; Middle Aged; Nitrobenzoates; Phenotype | 2019 |
Unusual first presentation of a metabolic disorder.
Topics: Cyclohexanones; Diet, Protein-Restricted; Disease Management; Fever; Humans; Infant; Male; Metabolic Diseases; Nitrobenzoates; Splenomegaly; Tonsillitis | 2019 |
LC-MS/MS study of the degradation processes of nitisinone and its by-products.
Topics: Chromatography, High Pressure Liquid; Cyclohexanones; Drug Stability; Gastric Juice; Humans; Hydrogen-Ion Concentration; Limit of Detection; Models, Biological; Molecular Structure; Nitrobenzoates; Saliva; Spectrometry, Mass, Electrospray Ionization; Tandem Mass Spectrometry; Tyrosinemias | 2019 |
Tocopherol biosynthesis in
Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Benzofurans; Cyclohexanones; Enzyme Inhibitors; Leishmania; Nitrobenzoates; Tocopherols | 2019 |
Live donor liver transplantation for type 1 tyrosinemia: An analysis of 15 patients.
Topics: Adolescent; Adult; Carcinoma, Hepatocellular; Child; Child, Preschool; Cyclohexanones; Databases, Factual; Female; Humans; Infant; Infant, Newborn; Liver Neoplasms; Liver Transplantation; Living Donors; Male; Neonatal Screening; Nitrobenzoates; Retrospective Studies; Treatment Outcome; Tyrosinemias | 2019 |
Interference of hydroxyphenylpyruvic acid, hydroxyphenyllactic acid and tyrosine on routine serum and urine clinical chemistry assays; implications for biochemical monitoring of patients with alkaptonuria treated with nitisinone.
Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Alkaptonuria; Cyclohexanones; Enzyme Inhibitors; Humans; Nitrobenzoates; Phenylpropionates; Phenylpyruvic Acids; Tyrosine | 2019 |
Efficacy of low dose nitisinone in the management of alkaptonuria.
Topics: Adult; Alkaptonuria; Child, Preschool; Cyclohexanones; Diet; Dose-Response Relationship, Drug; Female; Humans; Magnetic Resonance Spectroscopy; Male; Nitrobenzoates; Tyrosine; Young Adult | 2019 |
Senescence suppressed proliferation of host hepatocytes is precondition for liver repopulation.
Topics: Animals; Cell Cycle Checkpoints; Cell Proliferation; Cellular Senescence; Cyclohexanones; Hepatocytes; Hydrolases; Liver; Mice; Nitrobenzoates | 2019 |
Biomarkers of Micronutrients in Regular Follow-Up for Tyrosinemia Type 1 and Phenylketonuria Patients.
Topics: Adolescent; Adult; Aged; Amino Acids; Biomarkers; Biopterins; Child; Child, Preschool; Cyclohexanones; Diet, Protein-Restricted; Dietary Supplements; Enzyme Inhibitors; Female; Humans; Infant; Male; Micronutrients; Middle Aged; Nitrobenzoates; Nutritional Status; Phenylketonurias; Retrospective Studies; Risk Assessment; Risk Factors; Tyrosinemias; Vitamin B 12 Deficiency; Vitamin D Deficiency; Young Adult | 2019 |
Dietary restriction of tyrosine and phenylalanine lowers tyrosinemia associated with nitisinone therapy of alkaptonuria.
Topics: Alkaptonuria; Animals; Cyclohexanones; Diet, Protein-Restricted; Female; Humans; Male; Mice; Nitrobenzoates; Phenylalanine; Tyrosine; Tyrosinemias | 2020 |
Evolution of tyrosinemia type 1 disease in patients treated with nitisinone in Spain.
Topics: Adult; Child; Cognitive Dysfunction; Cyclohexanones; Delayed Diagnosis; Enzyme Inhibitors; Female; Follow-Up Studies; Humans; Infant, Newborn; Kidney Diseases; Male; Needs Assessment; Neonatal Screening; Nitrobenzoates; Obesity; Prognosis; Quality of Life; Retrospective Studies; Spain; Time-to-Treatment; Tyrosinemias | 2019 |
Tissue-specific FAH deficiency alters sleep-wake patterns and results in chronic tyrosinemia in mice.
Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Animals; Cells, Cultured; Circadian Rhythm; Cyclohexanones; Enzyme Inhibitors; Enzyme Stability; HEK293 Cells; Homozygote; Humans; Hydrolases; Kidney; Liver; Male; Mice; Mice, Inbred C57BL; Mutation; Nitrobenzoates; Organ Specificity; Sleep; Suprachiasmatic Nucleus; Tyrosinemias | 2019 |
The Effect of Various Doses of Phenylalanine Supplementation on Blood Phenylalanine and Tyrosine Concentrations in Tyrosinemia Type 1 Patients.
Topics: Adolescent; Adult; Child; Cyclohexanones; Dietary Supplements; Enzyme Inhibitors; Female; Heptanoates; Humans; Linear Models; Male; Nitrobenzoates; Phenylalanine; Tyrosine; Tyrosinemias; Young Adult | 2019 |
Emotional and behavioral problems, quality of life and metabolic control in NTBC-treated Tyrosinemia type 1 patients.
Topics: Adolescent; Adult; Child; Cyclohexanones; Humans; Male; Nitrobenzoates; Phenylalanine; Quality of Life; Tyrosine; Tyrosinemias; Young Adult | 2019 |
[Screening for hereditary tyrosinemia and genotype analysis in newborns].
Topics: Child; Cyclohexanones; Genotype; Humans; Infant; Infant, Newborn; Neonatal Screening; Nitrobenzoates; Tandem Mass Spectrometry; Tyrosinemias | 2019 |
Nitisinone causes acquired tyrosinosis in alkaptonuria.
Topics: Adult; Aged; Alkaptonuria; Amino Acid Metabolism, Inborn Errors; Animals; Cyclohexanones; Female; Humans; Male; Mice; Mice, Inbred BALB C; Middle Aged; Nitrobenzoates; Phenylalanine; Tyrosine; Young Adult | 2020 |
Late Development of Hepatocellular Carcinoma in Tyrosinemia Type 1 Despite Nitisinone (NTBC) Treatment.
Topics: Carcinoma, Hepatocellular; Cyclohexanones; Enzyme Inhibitors; Humans; Liver Neoplasms; Nitrobenzoates; Tyrosinemias | 2020 |
First Scandinavian case of successful pregnancy during nitisinone treatment for type 1 tyrosinemia.
Topics: Adolescent; Cyclohexanones; Enzyme Inhibitors; Female; Humans; Nitrobenzoates; Pregnancy; Pregnancy Complications; Pregnancy Outcome; Tyrosine; Tyrosinemias | 2020 |
Laboratory monitoring of patients with hereditary tyrosinemia type I.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Amino Acids; Biomarkers; Case-Control Studies; Child; Child, Preschool; Cyclohexanones; Female; Follow-Up Studies; Heptanoates; Humans; Infant; Infant, Newborn; Laboratories; Male; Middle Aged; Nitrobenzoates; Prognosis; Reference Standards; Specimen Handling; Tyrosinemias; Young Adult | 2020 |
Characterising the arthroplasty in spondyloarthropathy in a large cohort of eighty-seven patients with alkaptonuria.
Topics: Aged; Alkaptonuria; Arthroplasty; Cohort Studies; Cyclohexanones; Female; Humans; Linear Models; Male; Middle Aged; Nitrobenzoates; Ochronosis; Positron Emission Tomography Computed Tomography; Spondylarthropathies; United Kingdom | 2021 |
Oxidative Stress, Glutathione Metabolism, and Liver Regeneration Pathways Are Activated in Hereditary Tyrosinemia Type 1 Mice upon Short-Term Nitisinone Discontinuation.
Topics: Animals; Cyclohexanones; Disease Models, Animal; Glutathione; Humans; Hydrolases; Liver; Liver Regeneration; Medication Adherence; Mice; Mice, Knockout; NF-E2-Related Factor 2; Nitrobenzoates; Oxidative Stress; Oxidoreductases; Signal Transduction; Tyrosine; Tyrosinemias; Withholding Treatment | 2020 |
Hepatorenal Tyrosinaemia: Impact of a Simplified Diet on Metabolic Control and Clinical Outcome.
Topics: Adolescent; Austria; Child; Child, Preschool; Combined Modality Therapy; Cyclohexanones; Diet, Protein-Restricted; Dietary Proteins; Enzyme Inhibitors; Female; Germany; Humans; Male; Nitrobenzoates; Patient Compliance; Phenylalanine; Practice Guidelines as Topic; Prospective Studies; Retrospective Studies; Surveys and Questionnaires; Switzerland; Treatment Outcome; Tyrosine; Tyrosinemias; Young Adult | 2020 |
Characterizing the alkaptonuria joint and spine phenotype and assessing the effect of homogentisic acid lowering therapy in a large cohort of 87 patients.
Topics: Aged; Alkaptonuria; Cohort Studies; Cyclohexanones; Female; Homogentisic Acid; Humans; Joints; Linear Models; Male; Middle Aged; Nitrobenzoates; Ochronosis; Phenotype; Positron Emission Tomography Computed Tomography; Severity of Illness Index; Spine; United Kingdom | 2021 |
Repurposing the orphan drug nitisinone to control the transmission of African trypanosomiasis.
Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Animals; Bees; Cyclohexanones; Drug Repositioning; Female; Humans; Infection Control; Insecticides; Male; Metabolome; Mice; Models, Theoretical; Neglected Diseases; Nitrobenzoates; Orphan Drug Production; Rats; Rats, Wistar; Toxicity Tests; Trypanosomiasis, African; Tsetse Flies; Tyrosine | 2021 |
Nitisinone: two decades treating hereditary tyrosinaemia type 1.
Topics: Cyclohexanones; Humans; Nitrobenzoates; Tyrosinemias | 2021 |
Evaluating the aortic stenosis phenotype before and after the effect of homogentisic acid lowering therapy: Analysis of a large cohort of eighty-one alkaptonuria patients.
Topics: Adult; Aged; Alkaptonuria; Aortic Valve Stenosis; Cohort Studies; Cyclohexanones; Enzyme Inhibitors; Female; Humans; Male; Middle Aged; Nitrobenzoates; Phenotype; Severity of Illness Index | 2021 |
Influence of nitisinone and its metabolites on l-tyrosine metabolism in a model system.
Topics: Chromatography, Liquid; Cyclohexanones; Humans; Nitrobenzoates; Tandem Mass Spectrometry; Tyrosine | 2022 |
Preventive use of nitisinone in alkaptonuria.
Topics: Alkaptonuria; Cyclohexanones; Humans; Nitrobenzoates; Tyrosine | 2021 |
Liver Transplantation: A Safe and Definitive Alternative to Lifelong Nitisinone for Tyrosinemia Type 1.
Topics: Carcinoma, Hepatocellular; Child; Cyclohexanones; Humans; Liver Neoplasms; Liver Transplantation; Nitrobenzoates; Retrospective Studies; Treatment Outcome; Tyrosinemias | 2022 |
Nitisinone treatment during two pregnancies and breastfeeding in a woman with tyrosinemia type 1 - a case report.
Topics: Breast Feeding; Cyclohexanones; Female; Humans; Infant, Newborn; Nitrobenzoates; Pregnancy; Pregnancy Complications; Tyrosinemias; Young Adult | 2022 |
Evaluation of dynamic thiol/disulfide homeostasis in hereditary tyrosinemia type 1 patients.
Topics: Carcinoma, Hepatocellular; Cyclohexanones; Disulfides; Homeostasis; Humans; Liver Neoplasms; Nitrobenzoates; Oxidative Stress; Sulfhydryl Compounds; Tyrosine; Tyrosinemias | 2022 |
β-Cyclodextrin Derivative Grafted on Silica Gel Represents a New Polymeric Sorbent for Extracting Nitisinone from Model Physiological Fluids.
Topics: Adsorption; beta-Cyclodextrins; Cyclohexanones; Enzyme Inhibitors; Humans; Nitrobenzoates; Polymerization; Polymers; Silica Gel; Silicon Dioxide; Solid Phase Extraction | 2021 |
Hepatocellular carcinoma requiring liver transplantation in hereditary tyrosinemia type 1 despite nitisinone therapy and α1-fetoprotein normalization.
Topics: alpha-Fetoproteins; Carcinoma, Hepatocellular; Child; Cyclohexanones; Female; Humans; Liver Cirrhosis; Liver Neoplasms; Liver Transplantation; Nitrobenzoates; Tyrosinemias | 2022 |
Determinants of tyrosinaemia during nitisinone therapy in alkaptonuria.
Topics: Alkaptonuria; Brain Diseases, Metabolic, Inborn; Cyclohexanones; Homogentisic Acid; Humans; Nitrobenzoates; Phenylalanine; Phenylpropionates; Tyrosine; Tyrosinemias | 2022 |
[Alkaptonuria: Response to low-dose nitisinone in two patients with a new mutation].
Topics: Alkaptonuria; Cyclohexanones; Female; Humans; Male; Middle Aged; Mutation; Nitrobenzoates | 2022 |
Metabolic profiles and fingerprints for the investigation of the influence of nitisinone on the metabolism of the yeast Saccharomyces cerevisiae.
Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Cyclohexanones; Humans; Metabolome; Nitrobenzoates; Saccharomyces cerevisiae; Tandem Mass Spectrometry; Tyrosine | 2023 |