3-Methyl-2-oxovaleric acid

3-methyl-2-oxovaleric acid is 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). It has a role as a human metabolite. It is a 2-oxo monocarboxylic acid and a branched-chain keto acid. It is functionally related to a valeric acid. It is a conjugate acid of a 3-methyl-2-oxovalerate.