tyrosine and nitisinone

tyrosine has been researched along with nitisinone in 90 studies

Research

Studies (90)

Timeframe	Studies, this research(%)	All Research%
pre-1990	0 (0.00)	18.7374
1990's	23 (25.56)	18.2507
2000's	17 (18.89)	29.6817
2010's	35 (38.89)	24.3611
2020's	15 (16.67)	2.80

Authors

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	2
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
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
Ellis, MK; Gaskin, P; Lock, EA; McLean Provan, W; Robinson, M; Smith, LL	1
Eksborg, S; Hall, MG; Lumholtz, B; Provan, WM; Wilks, MF	1
Ahmad, S; Lueder, GT; Teckman, JH	1
Anderson, PD; Anikster, Y; Bernardini, I; Fitzpatrick, DL; Gahl, WA; Gerber, LH; Huizing, M; Introne, WJ; Murphey, MD; Perry, MB; Phornphutkul, C	1
Gissen, P; McKiernan, PJ; Preece, MA; Willshaw, HA	1
Dalton, TP; Dieter, MZ; Freshwater, SL; Miller, ML; Nebert, DW; Shertzer, HG	1
Lock, EA; Williams, RE	1
Berger, R; Grompe, M; Jacobs, SM; Klomp, LW; Luijerink, MC; Malingré, HE; van Beurden, EA; van den Berg, IE	1
Felitsyn, NM; Henderson, GN; James, MO; Stacpoole, PW	1
André, N; Jubin, V; Ovaert, C; Roquelaure, B	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
Finegold, M; Jorquera, R; Langlois, C; Shroads, AL; Stacpoole, PW; Tanguay, RM	1
McKiernan, PJ	1
Cohen, AF; van Bronswijk, H	1
Assmann, B; Herebian, D; Klee, D; Mayatepek, E; Spiekerkoetter, U; Thimm, E	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
Carton, D; Dan, B; De Laet, C; François, B; Goyens, PJ; Jaeken, J; Munoz, VT; Sokal, EM	1
Aldámiz-Echevarría, L; Couce, ML; Dalmau, J; del Toro, M; Pintos-Morell, G	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
Boenzi, S; Della Bona, ML; Dionisi-Vici, C; la Marca, G; Malvagia, S; Martinelli, D; Materazzi, S	1
Benoist, JF; Davit-Spraul, A; Garcia Segarra, N; Grenèche, MO; Imbard, A; Ogier de Baulny, H; Roche, S	1
Kassel, R; Rudnick, DA; Sprietsma, L	1
Arning, E; Bottiglieri, T; Gibson, KM; Grompe, M; Harding, CO; Winn, SR	1
Janovszky, A; Karg, E; László, A; Rózsa, M; Sallay, E; Tiszlavicz, L; Túri, S; Ugarte, M; Várkonyi, A; Wittmann, G	1
Qi, Z; Sun, R; Tian, Z; Wang, X; Wei, H	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
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
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
Curtis, S; Davison, AS; Devine, J; Gallagher, JA; Hughes, AT; Khedr, M; Milan, AM; Ranganath, LR; Usher, J	1
Morrow, G; Tanguay, RM	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
Das, AM; Meyer, U; van Rijn, M; van Spronsen, FJ	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
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
Adam, D	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
Cox, TF; Davison, AS; Gallagher, JA; Hughes, AT; Khedr, M; Milan, AM; Psarelli, EE; Ranganath, LR; Rhodes, NP; Rovensky, J	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
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
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
Äärelä, L; Hiltunen, P; Kurppa, K; Nevalainen, PI	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
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
Babiuch, M; Barchanska, H; Płonka, J	1
Heiner-Fokkema, MR; van Spronsen, FJ; Wolffenbuttel, BHR	1
Aktuglu Zeybek, AC; Cansever, MS; Erel, O; Iscan, HZ; Kiykim, E; Neselioglu, S; Zubarioglu, T	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
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
Barchanska, H; Kostina-Bednarz, M; Nowak, P; Płonka, J	1
Havranova, A; Imrich, R; Lukacova, O; Penesova, A; Radikova, Z; Ranganath, L; Sedlakova, J; Vlcek, M; Zanova, E; Zatkova, A	1
Bou-Gharios, G; Casimir, G; Claes, P; Colemonts-Vroninks, H; De Kock, J; Gallagher, JA; Goyens, P; Heymans, A; Hughes, JH; Lequeue, S; Marcélis, L; Martens, GA; Neuckermans, J; Vanhaecke, T	1

Reviews

13 review(s) available for tyrosine and nitisinone

Article	Year
Diagnosis and management of tyrosinemia type I. Current opinion in pediatrics, 1995, Volume: 7, Issue:6 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. Clinical and investigative medicine. Medecine clinique et experimentale, 1996, Volume: 19, Issue:5 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. Journal of inherited metabolic disease, 1998, Volume: 21, Issue:5 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). Journal of inherited metabolic disease, 1998, Volume: 21, Issue:5 Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Amino Acid Metabolism, Inborn Errors; Animals; Clinical Trials as Topic; Cyclohexanones; Enzyme Inhibitors; Humans; Nitrobenzoates; Tyrosine	1998
Old treatments for new insights and strategies: proposed management in adults and children with alkaptonuria. Journal of inherited metabolic disease, 2015, Volume: 38, Issue:5 Topics: Adult; Age Factors; Alkaptonuria; Antioxidants; Child; Cyclohexanones; Humans; Nitrobenzoates; Ochronosis; Phenylalanine; Tyrosine	2015
Biochemical and Clinical Aspects of Hereditary Tyrosinemia Type 1. Advances in experimental medicine and biology, 2017, Volume: 959 Topics: Cyclohexanones; Humans; Kidney; Liver; Liver Failure; Nitrobenzoates; Tyrosine; Tyrosinemias	2017
Neurological and Neuropsychological Problems in Tyrosinemia Type I Patients. Advances in experimental medicine and biology, 2017, Volume: 959 Topics: Cyclohexanones; Humans; Nervous System Diseases; Nitrobenzoates; Phenylalanine; Tyrosine; Tyrosinemias	2017
Hereditary Tyrosinemia Type 1 in Turkey. Advances in experimental medicine and biology, 2017, Volume: 959 Topics: Cyclohexanones; Humans; Hydrolases; Liver; Liver Diseases; Nitrobenzoates; Turkey; Tyrosine; Tyrosinemias	2017
From Weed Killer to Wonder Drug. Advances in experimental medicine and biology, 2017, Volume: 959 Topics: Animals; Biological Products; Cyclohexanones; Enzyme Inhibitors; Humans; Liver; Nitrobenzoates; Tyrosine; Tyrosinemias	2017
Dietary Considerations in Tyrosinemia Type I. Advances in experimental medicine and biology, 2017, Volume: 959 Topics: Cyclohexanones; Diet; Humans; Nitrobenzoates; Phenylalanine; Tyrosine; Tyrosinemias	2017
Alkaptonuria - Many questions answered, further challenges beckon. Annals of clinical biochemistry, 2020, Volume: 57, Issue:2 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. Paediatric drugs, 2019, Volume: 21, Issue:6 Topics: Cyclohexanones; Humans; Nitrobenzoates; Tyrosine; Tyrosinemias	2019
Efficacy of Phenylalanine- and Tyrosine-Restricted Diet in Alkaptonuria Patients on Nitisinone Treatment: Case Series and Review of Literature. Annals of nutrition & metabolism, 2022, Volume: 78, Issue:1 Topics: Adult; Alkaptonuria; Cyclohexanones; Diet; Dietary Proteins; Female; Humans; Male; Middle Aged; Nitrobenzoates; Phenylalanine; Tyrosine	2022

Trials

6 trial(s) available for tyrosine 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. British journal of clinical pharmacology, 2001, Volume: 52, Issue:2 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. Journal of inherited metabolic disease, 2003, Volume: 26, Issue:1 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. Molecular genetics and metabolism, 2011, Volume: 103, Issue:4 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. Developmental medicine and child neurology, 2011, Volume: 53, Issue:10 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 Annals of the rheumatic diseases, 2016, Volume: 75, Issue:2 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
Quantification of the flux of tyrosine pathway metabolites during nitisinone treatment of Alkaptonuria. Scientific reports, 2019, 07-11, Volume: 9, Issue:1 Topics: Adult; Alkaptonuria; Cyclohexanones; Female; Homogentisic Acid; Humans; Male; Middle Aged; Nitrobenzoates; Phenylalanine; Pigments, Biological; Tandem Mass Spectrometry; Tyrosine	2019

Other Studies

71 other study(ies) available for tyrosine and nitisinone

Article	Year
New treatment for tyrosinaemia. Lancet (London, England), 1992, Oct-03, Volume: 340, Issue:8823 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. Lancet (London, England), 1992, Oct-03, Volume: 340, Issue:8823 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. Nature genetics, 1995, Volume: 10, Issue:4 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. Journal of inherited metabolic disease, 1995, Volume: 18, Issue:2 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. Toxicology and applied pharmacology, 1995, Volume: 133, Issue:1 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. Journal of pediatric gastroenterology and nutrition, 1993, Volume: 17, Issue:3 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. Journal of neurology, neurosurgery, and psychiatry, 1993, Volume: 56, Issue:10 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. Nature genetics, 1996, Volume: 12, Issue:3 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. Human & experimental toxicology, 1996, Volume: 15, Issue:2 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). Journal of inherited metabolic disease, 1996, Volume: 19, Issue:2 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. Toxicology and applied pharmacology, 1996, Volume: 141, Issue:2 Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Animals; Aqueous Humor; Cyclohexanones; Eye; Liver; Male; Nitrobenzoates; Rats; Rats, Wistar; Tissue Distribution; Tyrosine	1996
[Late onset type I tyrosinemia]. Orvosi hetilap, 1997, Jul-13, Volume: 138, Issue:28 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. Human gene therapy, 1998, Feb-10, Volume: 9, Issue:3 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 Toxicology and applied pharmacology, 1998, Volume: 150, Issue:1 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. American journal of human genetics, 1998, Volume: 63, Issue:3 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. Journal of inherited metabolic disease, 1998, Volume: 21, Issue:5 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
[Favorable outcome of treatment with NTBC of acute liver insufficiency disclosing hereditary tyrosinemia type I]. Archives de pediatrie : organe officiel de la Societe francaise de pediatrie, 1999, Volume: 6, Issue:5 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. Journal of inherited metabolic disease, 1999, Volume: 22, Issue:3 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. Journal of inherited metabolic disease, 1999, Volume: 22, Issue:5 Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Adolescent; Amino Acid Metabolism, Inborn Errors; Chronic Disease; Cyclohexanones; Fatal Outcome; Humans; Male; Nitrobenzoates; Tyrosine	1999
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. Toxicology, 2000, Apr-03, Volume: 144, Issue:1-3 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
Long-term therapy with NTBC and tyrosine-restricted diet in a murine model of hereditary tyrosinemia type I. Molecular genetics and metabolism, 2002, Volume: 75, Issue:1 Topics: Animals; Cyclohexanones; Diet; Disease Models, Animal; Enzyme Inhibitors; Hydrolases; Mice; Mice, Knockout; Nitrobenzoates; Phenylalanine; Tyrosine; Tyrosinemias	2002
Nitisinone. Ntbc, Orfadin. Drugs in R&D, 2002, Volume: 3, Issue:2 Topics: Cyclohexanones; Humans; Nitrobenzoates; Tyrosine	2002
Corneal opacities associated with NTBC treatment. American journal of ophthalmology, 2002, Volume: 134, Issue:2 Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Child, Preschool; Cornea; Corneal Opacity; Cyclohexanones; Diet, Protein-Restricted; Enzyme Inhibitors; Female; Humans; Nitrobenzoates; Tyrosine; Tyrosinemias	2002
Natural history of alkaptonuria. The New England journal of medicine, 2002, Dec-26, Volume: 347, Issue:26 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
Pharmacological rescue of the 14CoS/14CoS mouse: hepatocyte apoptosis is likely caused by endogenous oxidative stress. Free radical biology & medicine, 2003, Aug-15, Volume: 35, Issue:4 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
D-serine-induced nephrotoxicity: possible interaction with tyrosine metabolism. Toxicology, 2004, Sep-01, Volume: 201, Issue:1-3 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. Kidney international, 2004, Volume: 66, Issue:3 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
Liquid chromatography-tandem mass spectrometry method for the simultaneous determination of delta-ALA, tyrosine and creatinine in biological fluids. Clinica chimica acta; international journal of clinical chemistry, 2004, Volume: 350, Issue:1-2 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. Journal of inherited metabolic disease, 2005, Volume: 28, Issue:1 Topics: Amino Acid Metabolism, Inborn Errors; Cardiomyopathy, Hypertrophic; Cyclohexanones; Echocardiography; Enzyme Inhibitors; Humans; Hypertrophy, Left Ventricular; Infant; Male; Nitrobenzoates; Tyrosine; Tyrosinemias	2005
Use of nitisinone in patients with alkaptonuria. Metabolism: clinical and experimental, 2005, Volume: 54, Issue:6 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. Pediatric blood & cancer, 2006, Volume: 46, Issue:4 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
Evaluation of dichloroacetate treatment in a murine model of hereditary tyrosinemia type 1. Biochemical pharmacology, 2006, May-28, Volume: 71, Issue:11 Topics: Animals; Cyclohexanones; Dichloroacetic Acid; Female; Glutathione; Glutathione Disulfide; Hydrolases; Kidney; Liver; Male; Mice; Nitrobenzoates; Oxidative Stress; Tyrosine; Tyrosinemias; Weight Loss	2006
Nitisinone in the treatment of hereditary tyrosinaemia type 1. Drugs, 2006, Volume: 66, Issue:6 Topics: Carcinoma, Hepatocellular; Cyclohexanones; Enzyme Inhibitors; Humans; Kidney Diseases; Liver Diseases; Liver Failure; Liver Neoplasms; Liver Transplantation; Monitoring, Physiologic; Nitrobenzoates; Tyrosine; Tyrosinemias	2006
[New drugs; nitisinone]. Nederlands tijdschrift voor geneeskunde, 2006, Nov-18, Volume: 150, Issue:46 Topics: Cyclohexanones; Enzyme Inhibitors; Humans; Nitrobenzoates; Tyrosine; Tyrosinemias	2006
Increase of CSF tyrosine and impaired serotonin turnover in tyrosinemia type I. Molecular genetics and metabolism, 2011, Volume: 102, Issue:2 Topics: Brain; Child; Cyclohexanones; Enzyme Inhibitors; Humans; Infant; Male; Nitrobenzoates; Psychomotor Performance; Serotonin; Tyrosine; Tyrosinemias	2011
Tyrosinemia type 1 in Spain: mutational analysis, treatment and long-term outcome. Pediatrics international : official journal of the Japan Pediatric Society, 2011, Volume: 53, Issue:6 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
Nitisinone improves eye and skin pigmentation defects in a mouse model of oculocutaneous albinism. The Journal of clinical investigation, 2011, Volume: 121, Issue:10 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. Journal of inherited metabolic disease, 2012, Volume: 35, Issue:2 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
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. Analytical chemistry, 2012, Jan-17, Volume: 84, Issue:2 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
Maternal and fetal tyrosinemia type I. Journal of inherited metabolic disease, 2010, Volume: 33 Suppl 3 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
Pregnancy in an NTBC-treated patient with hereditary tyrosinemia type I. Journal of pediatric gastroenterology and nutrition, 2015, Volume: 60, Issue:1 Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Adolescent; Combined Modality Therapy; Contraindications; Cyclohexanones; Enzyme Inhibitors; Female; Humans; Nitrobenzoates; Phenylalanine; Pregnancy; Pregnancy Complications; Term Birth; Tyrosine; Tyrosinemias	2015
Pharmacologic inhibition of L-tyrosine degradation ameliorates cerebral dopamine deficiency in murine phenylketonuria (PKU). Journal of inherited metabolic disease, 2014, Volume: 37, Issue:5 Topics: Amino Acids; Animals; Brain Chemistry; Cyclohexanones; Dopamine; Enzyme Inhibitors; Mice; Mice, Inbred C57BL; Mice, Knockout; Neurotransmitter Agents; Nitrobenzoates; Phenylketonurias; Tyrosine	2014
The fate of tyrosinaemic Hungarian patients before the NTBC aera. Ideggyogyaszati szemle, 2013, Nov-30, Volume: 66, Issue:11-12 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
Infiltrating neutrophils aggravate metabolic liver failure in fah-deficient mice. Liver international : official journal of the International Association for the Study of the Liver, 2015, Volume: 35, Issue:3 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
Serum markers in alkaptonuria: simultaneous analysis of homogentisic acid, tyrosine and nitisinone by liquid chromatography tandem mass spectrometry. Annals of clinical biochemistry, 2015, Volume: 52, Issue:Pt 5 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. Molecular genetics and metabolism, 2015, Volume: 114, Issue:3 Topics: Aldehydes; Alkaptonuria; Cell Line, Tumor; Cyclohexanones; Gastrointestinal Microbiome; Humans; Indoles; Mass Spectrometry; Metabolomics; Nitrobenzoates; Phenylpyruvic Acids; Symbiosis; Tryptophan; Tyrosine; Tyrosinemias	2015
Tyrosinemia type 1 and irreversible neurologic crisis after one month discontinuation of nitisone. Metabolic brain disease, 2016, Volume: 31, Issue:5 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. Journal of inherited metabolic disease, 2016, Volume: 39, Issue:5 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. Mathematical medicine and biology : a journal of the IMA, 2017, 09-01, Volume: 34, Issue:3 Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Animals; Computer Simulation; Cyclohexanones; Enzyme Inhibitors; Kinetics; Liver; Mathematical Concepts; Models, Animal; Models, Biological; Nitrobenzoates; Rats; Tyrosine; Tyrosinemias	2017
The effect of nitisinone on homogentisic acid and tyrosine: a two-year survey of patients attending the National Alkaptonuria Centre, Liverpool. Annals of clinical biochemistry, 2017, Volume: 54, Issue:3 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. Advances in experimental medicine and biology, 2017, Volume: 959 Topics: Child, Preschool; Cyclohexanones; Female; Humans; Infant; Japan; Liver; Liver Transplantation; Male; Nitrobenzoates; Phenylalanine; Tyrosine; Tyrosinemias	2017
Clinical and biochemical assessment of depressive symptoms in patients with Alkaptonuria before and after two years of treatment with nitisinone. Molecular genetics and metabolism, 2018, Volume: 125, Issue:1-2 Topics: Adolescent; Adult; Aged; Alkaptonuria; Cyclohexanones; Depression; Dopamine; Female; Humans; Hydroxyindoleacetic Acid; Male; Middle Aged; Nitrobenzoates; Tyrosine; Young Adult	2018
Minimal Efficacy of Nitisinone Treatment in a Novel Mouse Model of Oculocutaneous Albinism, Type 3. Investigative ophthalmology & visual science, 2018, 10-01, Volume: 59, Issue:12 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
A father's fight to help his sons - and fix clinical trials. Nature, 2019, Volume: 565, Issue:7738 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
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. Clinical biochemistry, 2019, Volume: 71 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. Molecular genetics and metabolism, 2019, Volume: 127, Issue:3 Topics: Adult; Alkaptonuria; Child, Preschool; Cyclohexanones; Diet; Dose-Response Relationship, Drug; Female; Humans; Magnetic Resonance Spectroscopy; Male; Nitrobenzoates; Tyrosine; Young Adult	2019
Dietary restriction of tyrosine and phenylalanine lowers tyrosinemia associated with nitisinone therapy of alkaptonuria. Journal of inherited metabolic disease, 2020, Volume: 43, Issue:2 Topics: Alkaptonuria; Animals; Cyclohexanones; Diet, Protein-Restricted; Female; Humans; Male; Mice; Nitrobenzoates; Phenylalanine; Tyrosine; Tyrosinemias	2020
The Effect of Various Doses of Phenylalanine Supplementation on Blood Phenylalanine and Tyrosine Concentrations in Tyrosinemia Type 1 Patients. Nutrients, 2019, Nov-18, Volume: 11, Issue:11 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. Orphanet journal of rare diseases, 2019, 12-04, Volume: 14, Issue:1 Topics: Adolescent; Adult; Child; Cyclohexanones; Humans; Male; Nitrobenzoates; Phenylalanine; Quality of Life; Tyrosine; Tyrosinemias; Young Adult	2019
Nitisinone causes acquired tyrosinosis in alkaptonuria. Journal of inherited metabolic disease, 2020, Volume: 43, Issue:5 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
First Scandinavian case of successful pregnancy during nitisinone treatment for type 1 tyrosinemia. Journal of pediatric endocrinology & metabolism : JPEM, 2020, May-26, Volume: 33, Issue:5 Topics: Adolescent; Cyclohexanones; Enzyme Inhibitors; Female; Humans; Nitrobenzoates; Pregnancy; Pregnancy Complications; Pregnancy Outcome; Tyrosine; Tyrosinemias	2020
Oxidative Stress, Glutathione Metabolism, and Liver Regeneration Pathways Are Activated in Hereditary Tyrosinemia Type 1 Mice upon Short-Term Nitisinone Discontinuation. Genes, 2020, 12-22, Volume: 12, Issue:1 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. Nutrients, 2020, Dec-31, Volume: 13, Issue:1 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
Repurposing the orphan drug nitisinone to control the transmission of African trypanosomiasis. PLoS biology, 2021, Volume: 19, Issue:1 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
Influence of nitisinone and its metabolites on l-tyrosine metabolism in a model system. Chemosphere, 2022, Volume: 286, Issue:Pt 1 Topics: Chromatography, Liquid; Cyclohexanones; Humans; Nitrobenzoates; Tandem Mass Spectrometry; Tyrosine	2022
Preventive use of nitisinone in alkaptonuria. Orphanet journal of rare diseases, 2021, 08-03, Volume: 16, Issue:1 Topics: Alkaptonuria; Cyclohexanones; Humans; Nitrobenzoates; Tyrosine	2021
Evaluation of dynamic thiol/disulfide homeostasis in hereditary tyrosinemia type 1 patients. Pediatric research, 2022, Volume: 92, Issue:2 Topics: Carcinoma, Hepatocellular; Cyclohexanones; Disulfides; Homeostasis; Humans; Liver Neoplasms; Nitrobenzoates; Oxidative Stress; Sulfhydryl Compounds; Tyrosine; Tyrosinemias	2022
Determinants of tyrosinaemia during nitisinone therapy in alkaptonuria. Scientific reports, 2022, 09-27, Volume: 12, Issue:1 Topics: Alkaptonuria; Brain Diseases, Metabolic, Inborn; Cyclohexanones; Homogentisic Acid; Humans; Nitrobenzoates; Phenylalanine; Phenylpropionates; Tyrosine; Tyrosinemias	2022
Metabolic profiles and fingerprints for the investigation of the influence of nitisinone on the metabolism of the yeast Saccharomyces cerevisiae. Scientific reports, 2023, 01-26, Volume: 13, Issue:1 Topics: 4-Hydroxyphenylpyruvate Dioxygenase; Cyclohexanones; Humans; Metabolome; Nitrobenzoates; Saccharomyces cerevisiae; Tandem Mass Spectrometry; Tyrosine	2023
Nutritional interventions for patients with alkaptonuria: A minireview. Endocrine regulations, 2023, Jan-01, Volume: 57, Issue:1 Topics: Alkaptonuria; Homogentisic Acid; Humans; Ochronosis; Tyrosine; Tyrosinemias	2023
Hereditary Tyrosinemia Type 1 Mice under Continuous Nitisinone Treatment Display Remnants of an Uncorrected Liver Disease Phenotype. Genes, 2023, 03-11, Volume: 14, Issue:3 Topics: Animals; Carcinoma, Hepatocellular; Liver Neoplasms; Mice; Phenotype; Tyrosine; Tyrosinemias	2023