has functional parent is conjugate acid of 2-hydroxy-3-methylpentanoic acid valeric acid (2R)-2-hydroxy monocarboxylic acid (2R,3S)-2-hydroxy-3-methylpentanoic acid (2R,3S)-2-hydroxy-3-methylpentanoic acid (2R,3S)-2-hydroxy-3-methylvaleric acid (2R,3S)-HMVA (2R,3S)-isoleucic acid (R,S)-isoleucic acid 132.079 132.15770 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). Beilstein:1721800 C6H12O3 CC[C@H](C)[C@@H](O)C(O)=O CHEBI:55536 HMVA InChI=1S/C6H12O3/c1-3-4(2)5(7)6(8)9/h4-5,7H,3H2,1-2H3,(H,8,9)/t4-,5+/m0/s1 PMID:1429566 RILPIWOPNGRASR-CRCLSJGQSA-N chebi_ontology (2R,3S)-2-hydroxy-3-methylpentanoate