SMILES: CC(C)C[C@H](C(=O)O)O
Description: The molecule is the (R)-enantiomer of 2-hydroxy-4-methylpentanoic acid. Found in patients with short-bowel syndrome (an inborn error of metabolism), and in maple syrup urine disease, MSUD. It is a (2R)-2-hydroxy monocarboxylic acid and a 2-hydroxy-4-methylvaleric acid. It is a conjugate acid of a (R)-2-hydroxy-4-methylpentanoate. It is an enantiomer of a (S)-2-hydroxy-4-methylpentanoic acid.