Nitisinone (BioDeep_00000033224)
human metabolite blood metabolite Chemicals and Drugs
代谢物信息卡片
2-(alpha,alpha,alpha-Trifluoro-2-nitro-p-tuluoyl)-1,3-cyclohexanedione
化学式: C14H10F3NO5 (329.0511046)
中文名称: 尼替西农
谱图信息:
最多检出来源 () 0%
分子结构信息
SMILES: C1CC(=O)C(C(=O)C1)C(=O)C2=C(C=C(C=C2)C(F)(F)F)[N+](=O)[O-]
InChI: InChI=1S/C14H10F3NO5/c15-14(16,17)7-4-5-8(9(6-7)18(22)23)13(21)12-10(19)2-1-3-11(12)20/h4-6,12H,1-3H2
描述信息
Nitisinone is only found in individuals that have used or taken this drug. It is a synthetic reversible inhibitor of 4-hydroxyphenylpyruvate dioxygenase. It is used in the treatment of hereditary tyrosinemia type 1. It is sold under the brand name Orfadin. [Wikipedia]Nitisinone is a competitive inhibitor of 4-hydroxyphenyl-pyruvate dioxygenase, an enzyme upstream of fumarylacetoacetate hydrolyase (FAH) in the tyrosine catabolic pathway. By inhibiting the normal catabolism of tyrosine in patients with hereditary tyrosinemia type 1 (HT-1), nitisinone prevents the accumulation of the catabolic intermediates maleylacetoacetate and fumarylacetoacetate.
A - Alimentary tract and metabolism > A16 - Other alimentary tract and metabolism products > A16A - Other alimentary tract and metabolism products > A16AX - Various alimentary tract and metabolism products
D004791 - Enzyme Inhibitors
C471 - Enzyme Inhibitor
同义名列表
11 个代谢物同义名
2-(alpha,alpha,alpha-Trifluoro-2-nitro-p-tuluoyl)-1,3-cyclohexanedione; 2-(2-Nitro-4-(trifluoromethyl)benzoyl)cyclohexane-1,3-dione; 2-[2-nitro-4-(trifluoromethyl)benzoyl]cyclohexane-1,3-dione; 2-(a,a,a-Trifluoro-2-nitro-p-tuluoyl)-1,3-cyclohexanedione; 2-(Α,α,α-trifluoro-2-nitro-p-tuluoyl)-1,3-cyclohexanedione; 2-(2-Nitro-4-trifluoromethylbenzoyl)-1,3-cyclohexanedione; Nitisinonum; Nitisinone; Nitisinona; NTBC CPD; Orfadin
数据库引用编号
8 个数据库交叉引用编号
- ChEBI: CHEBI:50378
- PubChem: 115355
- HMDB: HMDB0014492
- DrugBank: DB00348
- ChEMBL: CHEMBL1337
- Wikipedia: Nitisinone
- chemspider: 103195
- CAS: 104206-65-7
分类词条
相关代谢途径
Reactome(0)
BioCyc(0)
PlantCyc(0)
代谢反应
0 个相关的代谢反应过程信息。
Reactome(0)
BioCyc(0)
WikiPathways(0)
Plant Reactome(0)
INOH(0)
PlantCyc(0)
COVID-19 Disease Map(0)
PathBank(0)
PharmGKB(0)
1 个相关的物种来源信息
在这里通过桑基图来展示出与当前的这个代谢物在我们的BioDeep知识库中具有相关联信息的其他代谢物。在这里进行关联的信息来源主要有：
- PubMed: 来源于PubMed文献库中的文献信息，我们通过自然语言数据挖掘得到的在同一篇文献中被同时提及的相关代谢物列表，这个列表按照代谢物同时出现的文献数量降序排序，取前10个代谢物作为相关研究中关联性很高的代谢物集合展示在桑基图中。
- NCBI Taxonomy: 通过文献数据挖掘，得到的代谢物物种来源信息关联。这个关联信息同样按照出现的次数降序排序，取前10个代谢物作为高关联度的代谢物集合展示在桑吉图上。
- Chemical Taxonomy: 在物质分类上处于同一个分类集合中的其他代谢物
- Chemical Reaction: 在化学反应过程中，存在为当前代谢物相关联的生化反应过程中的反应底物或者反应产物的关联代谢物信息。
点击图上的相关代谢物的名称，可以跳转到相关代谢物的信息页面。
文献列表
- Joanna Płonka, Monika Babiuch, Hanna Barchanska. Influence of nitisinone and its metabolites on l-tyrosine metabolism in a model system.
Chemosphere.
2022 Jan; 286(Pt 1):131592. doi:
10.1016/j.chemosphere.2021.131592. [PMID: 34311397] - Pelin Teke Kisa, Semra Eroglu Erkmen, Hilal Bahceci, Zumrut Arslan Gulten, Ayca Aydogan, Ozge Kamer Karalar Pekuz, Tuba Yuce Inel, Taylan Ozturk, Sezer Uysal, Nur Arslan. Efficacy of Phenylalanine- and Tyrosine-Restricted Diet in Alkaptonuria Patients on Nitisinone Treatment: Case Series and Review of Literature.
Annals of nutrition & metabolism.
2022; 78(1):48-60. doi:
10.1159/000519813. [PMID: 34736252] - Bruce H R Wolffenbuttel, M Rebecca Heiner-Fokkema, Francjan J van Spronsen. Preventive use of nitisinone in alkaptonuria.
Orphanet journal of rare diseases.
2021 08; 16(1):343. doi:
10.1186/s13023-021-01977-0. [PMID: 34344451] - L R Ranganath, T Heseltine, M Khedr, M F Fisher. 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.
Molecular genetics and metabolism.
2021 07; 133(3):324-331. doi:
10.1016/j.ymgme.2021.05.007. [PMID: 34059444] - Ute Spiekerkoetter, Maria L Couce, Anibh M Das, Corinne de Laet, Carlo Dionisi-Vici, Allan M Lund, Manuel Schiff, Marco Spada, Erik Sparve, Johan Szamosi, Roshni Vara, Mattias Rudebeck. Long-term safety and outcomes in hereditary tyrosinaemia type 1 with nitisinone treatment: a 15-year non-interventional, multicentre study.
The lancet. Diabetes & endocrinology.
2021 07; 9(7):427-435. doi:
10.1016/s2213-8587(21)00092-9. [PMID: 34023005] - Lakshminarayan R Ranganath, Milad Khedr, Sobhan Vinjamuri, James A Gallagher. Characterizing the alkaptonuria joint and spine phenotype and assessing the effect of homogentisic acid lowering therapy in a large cohort of 87 patients.
Journal of inherited metabolic disease.
2021 05; 44(3):666-676. doi:
10.1002/jimd.12363. [PMID: 33452825] - Lakshminarayan R Ranganath, James A Gallagher, John Davidson, Sobhan Vinjamuri. Characterising the arthroplasty in spondyloarthropathy in a large cohort of eighty-seven patients with alkaptonuria.
Journal of inherited metabolic disease.
2021 05; 44(3):656-665. doi:
10.1002/jimd.12340. [PMID: 33314212] - Friederike Bärhold, Uta Meyer, Anne-Kathrin Neugebauer, Eva Maria Thimm, Dinah Lier, Stefanie Rosenbaum-Fabian, Ulrike Och, Anna Fekete, Dorothea Möslinger, Carmen Rohde, Skadi Beblo, Michel Hochuli, Nina Bogovic, Vanessa Korpel, Stephan Vom Dahl, Sebene Mayorandan, Aleksandra Fischer, Peter Freisinger, Katharina Dokoupil, Margret Heddrich-Ellerbrok, Monika Jörg-Streller, Agnes van Teeffelen-Heithoff, Janina Lahl, Anibh Martin Das. Hepatorenal Tyrosinaemia: Impact of a Simplified Diet on Metabolic Control and Clinical Outcome.
Nutrients.
2020 Dec; 13(1):. doi:
10.3390/nu13010134. [PMID: 33396520] - Lakshminarayan R Ranganath, Eftychia Eirini Psarelli, Jean-Baptiste Arnoux, Daniela Braconi, Michael Briggs, Anders Bröijersén, Nadia Loftus, Helen Bygott, Trevor F Cox, Andrew S Davison, Jane P Dillon, Michael Fisher, Richard FitzGerald, Federica Genovese, Helena Glasova, Anthony K Hall, Andrew T Hughes, Juliette H Hughes, Richard Imrich, Jonathan C Jarvis, Milad Khedr, Dinny Laan, Kim-Hanh Le Quan Sang, Emily Luangrath, Ol'ga Lukáčová, Anna M Milan, Alpesh Mistry, Vanda Mlynáriková, Brendan P Norman, Birgitta Olsson, Nicholas P Rhodes, Jozef Rovenský, Mattias Rudebeck, Annalisa Santucci, Ella Shweihdi, Ciarán Scott, Jana Sedláková, Nicolas Sireau, Roman Stančík, Johan Szamosi, Sophie Taylor, Christa van Kan, Sobhan Vinjamuri, Eva Vrtíková, Chris Webb, Elizabeth West, Elizabeth Záňová, Andrea Zatkova, James A Gallagher. Efficacy and safety of once-daily nitisinone for patients with alkaptonuria (SONIA 2): an international, multicentre, open-label, randomised controlled trial.
The lancet. Diabetes & endocrinology.
2020 09; 8(9):762-772. doi:
10.1016/s2213-8587(20)30228-x. [PMID: 32822600] - Linnea Äärelä, Pasi I Nevalainen, Kalle Kurppa, Pauliina Hiltunen. First Scandinavian case of successful pregnancy during nitisinone treatment for type 1 tyrosinemia.
Journal of pediatric endocrinology & metabolism : JPEM.
2020 May; 33(5):661-664. doi:
10.1515/jpem-2019-0540. [PMID: 32238608] - Shuzhang Yang, Sandra M Siepka, Kimberly H Cox, Vivek Kumar, Marleen de Groot, Yogarany Chelliah, Jun Chen, Benjamin Tu, Joseph S Takahashi. Tissue-specific FAH deficiency alters sleep-wake patterns and results in chronic tyrosinemia in mice.
Proceedings of the National Academy of Sciences of the United States of America.
2019 10; 116(44):22229-22236. doi:
10.1073/pnas.1904485116. [PMID: 31611405] - Willem G van Ginkel, Danique van Vliet, Els van der Goot, Martijn H J R Faassen, Arndt Vogel, M Rebecca Heiner-Fokkema, Eddy A van der Zee, Francjan J van Spronsen. Blood and Brain Biochemistry and Behaviour in NTBC and Dietary Treated Tyrosinemia Type 1 Mice.
Nutrients.
2019 Oct; 11(10):. doi:
10.3390/nu11102486. [PMID: 31623189] - S L Curtis, B P Norman, A M Milan, J A Gallagher, B Olsson, L R Ranganath, N B Roberts. 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 Sep; 71(?):24-30. doi:
10.1016/j.clinbiochem.2019.06.010. [PMID: 31228435] - María Luz Couce, Paula Sánchez-Pintos, Luís Aldámiz-Echevarría, Isidro Vitoria, Victor Navas, Elena Martín-Hernández, Camila García-Volpe, Guillem Pintos, Luis Peña-Quintana, Tomás Hernández, David Gil, Félix Sánchez-Valverde, María Bueno, Iria Roca, Encarna López-Ruzafa, Carmen Díaz-Fernández. Evolution of tyrosinemia type 1 disease in patients treated with nitisinone in Spain.
Medicine.
2019 Sep; 98(39):e17303. doi:
10.1097/md.0000000000017303. [PMID: 31574857] - Hanna Barchanska, Rafał Rola, Wojciech Szczepankiewicz, Marta Mrachacz. LC-MS/MS study of the degradation processes of nitisinone and its by-products.
Journal of pharmaceutical and biomedical analysis.
2019 Jul; 171(?):15-21. doi:
10.1016/j.jpba.2019.03.046. [PMID: 30959315] - A M Milan, A T Hughes, A S Davison, M Khedr, J Rovensky, E E Psarelli, T F Cox, N P Rhodes, J A Gallagher, L R Ranganath. Quantification of the flux of tyrosine pathway metabolites during nitisinone treatment of Alkaptonuria.
Scientific reports.
2019 07; 9(1):10024. doi:
10.1038/s41598-019-46033-x. [PMID: 31296884] - Natacha Sloboda, Arnaud Wiedemann, Marc Merten, Amerh Alqahtani, Elise Jeannesson, Alain Blum, Sophie Henn-Ménétré, Jean-Louis Guéant, Emeline Renard, François Feillet. Efficacy of low dose nitisinone in the management of alkaptonuria.
Molecular genetics and metabolism.
2019 07; 127(3):184-190. doi:
10.1016/j.ymgme.2019.06.006. [PMID: 31235217] - A S Davison, N Strittmatter, H Sutherland, A T Hughes, J Hughes, G Bou-Gharios, A M Milan, R J A Goodwin, L R Ranganath, J A Gallagher. Assessing the effect of nitisinone induced hypertyrosinaemia on monoamine neurotransmitters in brain tissue from a murine model of alkaptonuria using mass spectrometry imaging.
Metabolomics : Official journal of the Metabolomic Society.
2019 04; 15(5):68. doi:
10.1007/s11306-019-1531-4. [PMID: 31037385] - Brendan P Norman, Andrew S Davison, Gordon A Ross, Anna M Milan, Andrew T Hughes, Hazel Sutherland, Jonathan C Jarvis, Norman B Roberts, James A Gallagher, Lakshminarayan R Ranganath. A Comprehensive LC-QTOF-MS Metabolic Phenotyping Strategy: Application to Alkaptonuria.
Clinical chemistry.
2019 04; 65(4):530-539. doi:
10.1373/clinchem.2018.295345. [PMID: 30782595] - Gunilla Huledal, Birgitta Olsson, Kristin Önnestam, Per Dalén, Daniel Lindqvist, Matthias Kruse, Anders Bröijersén. 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.
European journal of clinical pharmacology.
2019 Mar; 75(3):313-320. doi:
10.1007/s00228-018-2581-7. [PMID: 30443705] - David Adam. A father's fight to help his sons - and fix clinical trials.
Nature.
2019 01; 565(7738):148-151. doi:
10.1038/d41586-019-00035-x. [PMID: 30626961] - Ighovie F Onojafe, Lucyanne H Megan, Madeline G Melch, Joseph O Aderemi, Ramakrishna P Alur, Mones S Abu-Asab, Chi-Chao Chan, Isa M Bernardini, Jessica S Albert, Tiziana Cogliati, David R Adams, Brian P Brooks. Minimal Efficacy of Nitisinone Treatment in a Novel Mouse Model of Oculocutaneous Albinism, Type 3.
Investigative ophthalmology & visual science.
2018 10; 59(12):4945-4952. doi:
10.1167/iovs.16-20293. [PMID: 30347088] - A S Davison, J A Harrold, G Hughes, B P Norman, J Devine, J Usher, A T Hughes, M Khedr, J A Gallagher, A M Milan, Halford J C G, L R Ranganath. 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 09; 125(1-2):135-143. doi:
10.1016/j.ymgme.2018.07.008. [PMID: 30049652] - Jonas Sundberg, Flemming Wibrand, Allan Meldgaard Lund, Mette Christensen. Simultaneous quantification of succinylacetone and nitisinone for therapeutic drug monitoring in the treatment of Tyrosinemia type 1.
Journal of chromatography. B, Analytical technologies in the biomedical and life sciences.
2018 Jan; 1072(?):259-266. doi:
10.1016/j.jchromb.2017.11.031. [PMID: 29195145] - John P Ward, Joanne L Dunster, Gianne Derks, Pratibha Mistry, José D Salazar. 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; 34(3):335-390. doi:
10.1093/imammb/dqw006. [PMID: 27305933] - Anna M Milan, Andrew T Hughes, Andrew S Davison, Jean Devine, Jeannette Usher, Sarah Curtis, Milad Khedr, James A Gallagher, Lakshminarayan R Ranganath. 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 May; 54(3):323-330. doi:
10.1177/0004563217691065. [PMID: 28081634] - Geneviève Morrow, Robert M Tanguay. Biochemical and Clinical Aspects of Hereditary Tyrosinemia Type 1.
Advances in experimental medicine and biology.
2017; 959(?):9-21. doi:
10.1007/978-3-319-55780-9_2. [PMID: 28755181] - Fernando Alvarez, Suzanne Atkinson, Manon Bouchard, Catherine Brunel-Guitton, Daniela Buhas, Jean-François Bussières, Josée Dubois, Daphna Fenyves, Paul Goodyer, Martyne Gosselin, Ugur Halac, Patrick Labbé, Rachel Laframboise, Bruno Maranda, Serge Melançon, Aicha Merouani, Grant A Mitchell, John Mitchell, Guy Parizeault, Luc Pelletier, Véronique Phan, Jean-François Turcotte. The Québec NTBC Study.
Advances in experimental medicine and biology.
2017; 959(?):187-195. doi:
10.1007/978-3-319-55780-9_17. [PMID: 28755196] - Arianna Maiorana, Carlo Dionisi-Vici. NTBC and Correction of Renal Dysfunction.
Advances in experimental medicine and biology.
2017; 959(?):93-100. doi:
10.1007/978-3-319-55780-9_8. [PMID: 28755187] - Grant A Mitchell, Hao Yang. Remaining Challenges in the Treatment of Tyrosinemia from the Clinician's Viewpoint.
Advances in experimental medicine and biology.
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Clinical chemistry.
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Journal of inherited metabolic disease.
2016 09; 39(5):673-682. doi:
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Irish medical journal.
2016 Jun; 109(6):426. doi:
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Indian journal of gastroenterology : official journal of the Indian Society of Gastroenterology.
2016 May; 35(3):229-31. doi:
10.1007/s12664-016-0650-3. [PMID: 27109516] - Marcella Laschi, Giulia Bernardini, Elena Dreassi, Lia Millucci, Michela Geminiani, Daniela Braconi, Barbara Marzocchi, Maurizio Botta, Fabrizio Manetti, Annalisa Santucci. Inhibition of para-Hydroxyphenylpyruvate Dioxygenase by Analogues of the Herbicide Nitisinone As a Strategy to Decrease Homogentisic Acid Levels, the Causative Agent of Alkaptonuria.
ChemMedChem.
2016 Apr; 11(7):674-8. doi:
10.1002/cmdc.201500578. [PMID: 26947423] - Lakshminarayan R Ranganath, Anna M Milan, Andrew T Hughes, John J Dutton, Richard Fitzgerald, Michael C Briggs, Helen Bygott, Eftychia E Psarelli, Trevor F Cox, James A Gallagher, Jonathan C Jarvis, Christa van Kan, Anthony K Hall, Dinny Laan, Birgitta Olsson, Johan Szamosi, Mattias Rudebeck, Torbjörn Kullenberg, Arvid Cronlund, Lennart Svensson, Carin Junestrand, Hana Ayoob, Oliver G Timmis, Nicolas Sireau, Kim-Hanh Le Quan Sang, Federica Genovese, Daniela Braconi, Annalisa Santucci, Martina Nemethova, Andrea Zatkova, Judith McCaffrey, Peter Christensen, Gordon Ross, Richard Imrich, Jozef Rovensky. 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 excretion in patients with alkaptonuria after 4 weeks of treatment.
Annals of the rheumatic diseases.
2016 Feb; 75(2):362-7. doi:
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Annals of clinical biochemistry.
2015 Sep; 52(Pt 5):597-605. doi:
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Pediatrics international : official journal of the Japan Pediatric Society.
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Molecular genetics and metabolism.
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Liver international : official journal of the International Association for the Study of the Liver.
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Molecular genetics and metabolism.
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Journal of inherited metabolic disease.
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Orphanet journal of rare diseases.
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Annals of hepatology.
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The Journal of pediatrics.
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Annals of the rheumatic diseases.
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Ideggyogyaszati szemle.
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BMC research notes.
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Journal of inherited metabolic disease.
2013 Jan; 36(1):15-20. doi:
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BMJ case reports.
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Journal of inherited metabolic disease.
2012 Sep; 35(5):831-6. doi:
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Molecular genetics and metabolism.
2012 Sep; 107(1-2):49-54. doi:
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Journal of chromatographic science.
2012 May; 50(5):446-9. doi:
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Journal of human nutrition and dietetics : the official journal of the British Dietetic Association.
2012 Apr; 25(2):111-6. doi:
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Journal of inherited metabolic disease.
2012 Mar; 35(2):263-8. doi:
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Analytical chemistry.
2012 Jan; 84(2):1184-8. doi:
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Pediatrics international : official journal of the Japan Pediatric Society.
2011 Dec; 53(6):985-9. doi:
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The Journal of clinical investigation.
2011 Oct; 121(10):3828-31. doi:
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The Journal of clinical investigation.
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2011 Oct; 94(10):1276-80. doi:
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Molecular genetics and metabolism.
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World journal of pediatrics : WJP.
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Journal of chromatography. B, Analytical technologies in the biomedical and life sciences.
2011 Apr; 879(11-12):671-6. doi:
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Molecular genetics and metabolism.
2011 Feb; 102(2):122-5. doi:
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Clinica chimica acta; international journal of clinical chemistry.
2011 Jan; 412(1-2):134-8. doi:
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Journal of inherited metabolic disease.
2010 Dec; 33 Suppl 3(?):S507-10. doi:
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Dermatology online journal.
2010 Nov; 16(11):6. doi:
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Journal of medicine and life.
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Biochemistry.
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European journal of pediatrics.
2010 Jun; 169(6):689-93. doi:
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Journal of gastroenterology and hepatology.
2010 May; 25(5):1002-8. doi:
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Talanta.
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Rapid communications in mass spectrometry : RCM.
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Metabolism: clinical and experimental.
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Casopis lekaru ceskych.
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Orphanet journal of rare diseases.
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Cancer cell.
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Acta biochimica Polonica.
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Molecular therapy : the journal of the American Society of Gene Therapy.
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Toxicology and applied pharmacology.
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Biochemical pharmacology.
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Kidney international.
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