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The Discovery of the Mode of Action of Nitisinone

Authors :
Edward A. Lock
Source :
Metabolites, Vol 12, Iss 10, p 902 (2022)
Publication Year :
2022
Publisher :
MDPI AG, 2022.

Abstract

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.

Details

Language :
English
ISSN :
22181989
Volume :
12
Issue :
10
Database :
Directory of Open Access Journals
Journal :
Metabolites
Publication Type :
Academic Journal
Accession number :
edsdoj.7f0692ba94348779e41e1910d3aa8ab
Document Type :
article
Full Text :
https://doi.org/10.3390/metabo12100902