alpha-ketoisovalerate

alpha-Ketoisovalerate (also known as alpha-ketoisovaleric acid) is a short-chain keto acid and an intermediate in the metabolism of the branched-chain amino acid valine. It is a colorless organic compound, typically encountered as its conjugate base, ketoisovalerate. In human physiology, it arises from the incomplete breakdown of valine and can accumulate abnormally in certain metabolic disorders such as maple syrup urine disease (MSUD), where it acts as a neurotoxin and metabotoxin at high concentrations[1][7]. Mechanistically, elevated levels can inhibit key enzymes involved in gluconeogenesis, the urea cycle, and pyruvate metabolism by direct or indirect effects on enzyme activity[5][8]. In bacteria such as Mycobacterium tuberculosis, it serves as a substrate for 2-isopropylmalate synthase (leuA gene product), catalyzing steps in leucine biosynthesis[2]. Industrially and biotechnologically, genetic engineering has enabled its use for biofuel production via conversion to isobutanol[7].

Other names

ketoisovalerate