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ChEBI

(2R,3S)-2-hydroxy-3-methylpentanoic acid

Term ID
CHEBI:55536
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).
References
Ontology
ChEBI  ( EBI )
Relationships
is a type of
has_functional_parent
inverse is_conjugate_base_of
is_conjugate_acid_of
Phenotype
Phenotype resulting from (2R,3S)-2-hydroxy-3-methylpentanoic acid
Phenotype where environments contain (2R,3S)-2-hydroxy-3-methylpentanoic acid
Phenotype modified by environments containing (2R,3S)-2-hydroxy-3-methylpentanoic acid
Human Disease / Model Data