ChEBI: (2R,3S)-2-hydroxy-3-methylpentanoic acid is a valeric acid derivative having an (R)-hydroxy substituent at the 2-position and an (S)-methyl substituent at the 3-position; one of 4 steroisomers of 2-hydroxy-3-methylpentanoic acid, generated by isoleucine metabolism. Found at significantly higher levels than normal in patients with maple syrup urine disease (MSUD). It is a (2R)-2-hydroxy monocarboxylic acid and a 2-hydroxy-3-methylpentanoic acid. It is functionally related to a valeric acid. It is a conjugate acid of a (2R,3S)-2-hydroxy-3-methylpentanoate.
