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The Discovery of the Mode of Action of Nitisinone

This review briefly discusses the discovery of the mode of action of the triketone herbicide, 2-(2-nitro-4-trifluormethylbenzoyl)-1,3-cyclohexanedione and its use as a drug Nitisinone for the treatment of inborn errors of tyrosine metabolism. Nitisinone is a potent reversible tight-binding inhibitor...

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Detalles Bibliográficos
Autor principal: Lock, Edward A.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9609752/
https://www.ncbi.nlm.nih.gov/pubmed/36295804
http://dx.doi.org/10.3390/metabo12100902
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author Lock, Edward A.
author_facet Lock, Edward A.
author_sort Lock, Edward A.
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description This review briefly discusses the discovery of the mode of action of the triketone herbicide, 2-(2-nitro-4-trifluormethylbenzoyl)-1,3-cyclohexanedione and its use as a drug Nitisinone for the treatment of inborn errors of tyrosine metabolism. Nitisinone is a potent reversible tight-binding inhibitor of the enzyme 4-hydroxyphenylpyruvate dioxygenase, involved in the catabolism of the amino acid tyrosine. Nitisinone is used to treat the rare disease hereditary tyrosinaemia type 1 where the last enzyme in the breakdown of tyrosine, fumarylacetoacetase is deficient. Nitisinone is also used to treat patients with alkaptonuria where the enzyme homogentisic acid oxidase is deficient. Articles in this issue discuss metabolites of tyrosine catabolism in healthy patients and those with alkaptonuria.
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spelling pubmed-96097522022-10-28 The Discovery of the Mode of Action of Nitisinone Lock, Edward A. Metabolites Review This review briefly discusses the discovery of the mode of action of the triketone herbicide, 2-(2-nitro-4-trifluormethylbenzoyl)-1,3-cyclohexanedione and its use as a drug Nitisinone for the treatment of inborn errors of tyrosine metabolism. Nitisinone is a potent reversible tight-binding inhibitor of the enzyme 4-hydroxyphenylpyruvate dioxygenase, involved in the catabolism of the amino acid tyrosine. Nitisinone is used to treat the rare disease hereditary tyrosinaemia type 1 where the last enzyme in the breakdown of tyrosine, fumarylacetoacetase is deficient. Nitisinone is also used to treat patients with alkaptonuria where the enzyme homogentisic acid oxidase is deficient. Articles in this issue discuss metabolites of tyrosine catabolism in healthy patients and those with alkaptonuria. MDPI 2022-09-25 /pmc/articles/PMC9609752/ /pubmed/36295804 http://dx.doi.org/10.3390/metabo12100902 Text en © 2022 by the author. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Review
Lock, Edward A.
The Discovery of the Mode of Action of Nitisinone
title The Discovery of the Mode of Action of Nitisinone
title_full The Discovery of the Mode of Action of Nitisinone
title_fullStr The Discovery of the Mode of Action of Nitisinone
title_full_unstemmed The Discovery of the Mode of Action of Nitisinone
title_short The Discovery of the Mode of Action of Nitisinone
title_sort discovery of the mode of action of nitisinone
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9609752/
https://www.ncbi.nlm.nih.gov/pubmed/36295804
http://dx.doi.org/10.3390/metabo12100902
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