Term Name: | (2R,3S)-2-hydroxy-3-methylpentanoic acid |
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Synonyms: | (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, HMVA |
Definition: | 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). |
Ontology: | ChEBI [CHEBI:55536] ( EBI ) |