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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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Formato: | Online Artículo Texto |
Lenguaje: | English |
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MDPI
2022
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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. |
collection | PubMed |
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. |
format | Online Article Text |
id | pubmed-9609752 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
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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