ChEBI: 2-hydroxy-4-methylvaleric acid is a valeric acid derivative having a hydroxy substituent at the 2-position and a methyl substituent at the 4-position; an alpha-hydroxy analogue of leucine. A bacterial metabolite, it has also been isolated from amniotic fluid, was found in a patient with dihydrolipoyl dehydrogenase deficiency and is present in the urine of patients with short bowel syndrome. It has a role as a metabolite. It is a 2-hydroxy fatty acid and a branched-chain fatty acid. It is functionally related to a valeric acid. It is a conjugate acid of a 2-hydroxy-4-methylvalerate.
