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).
Stars
This entity has been manually annotated by the ChEBI Team.
(2R,3S)-2-hydroxy-3-methylpentanoic acid
(CHEBI:55536)
has functional parent
valeric acid
(CHEBI:17418)
(2R,3S)-2-hydroxy-3-methylpentanoic acid
(CHEBI:55536)
is a
(2R)-2-hydroxy monocarboxylic acid
(CHEBI:17893)
(2R,3S)-2-hydroxy-3-methylpentanoic acid
(CHEBI:55536)
is a
2-hydroxy-3-methylpentanoic acid
(CHEBI:133083)
(2R,3S)-2-hydroxy-3-methylpentanoic acid
(CHEBI:55536)
is conjugate acid of
(2R,3S)-2-hydroxy-3-methylpentanoate
(CHEBI:55537)
Incoming
(2R,3S)-2-hydroxy-3-methylpentanoate
(CHEBI:55537)
is conjugate base of
(2R,3S)-2-hydroxy-3-methylpentanoic acid
(CHEBI:55536)