Term Name: | 3-methyl-2-oxovaleric acid |
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Synonyms: | 2-Oxo-3-methyl-n-valeric acid, 2-Oxo-3-methylpentanoic acid, 2-Oxo-3-methylvalerate, 2-Oxo-3-methylvaleric acid, 2-Oxoisoleucine, 2-oxokolavenic acid, 3-ethyl-3-methylpyruvic acid, 3-Methyl-2-oxopentanoic acid, 3-methyl-2-oxovaleric acid, alpha-keto-beta-methyl-n-valeric acid, alpha-keto-beta-methylvaleric acid, alpha-oxo-beta-methyl-n-valeric acid, alpha-oxo-beta-methylvaleric acid, ketoisoleucine |
Definition: | A 2-oxo monocarboxylic acid that is valeric acid carrying oxo- and methyl substituents at C-2 and C-3, respectively. An alpha-keto acid analogue and metabolite of isoleucine in man, animals and bacteria. Used as a clinical marker for maple syrup urine disease (MSUD). |
Ontology: | ChEBI [CHEBI:35932] ( EBI ) |