3-Hydroxy-2-methylbutanoic acid is a short-chain carboxylic acid and a normal urinary metabolite produced via isoleucine catabolism, fatty acid β-oxidation and ketogenesis. 3-Hydroxy-2-methylbutanoic acid exists as an acylated residue in resin glycosides derived from plants of the Ipomoea genus. 3-Hydroxy-2-methylbutanoic acid can be used in studies related to β-ketothiolase deficiency.
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