4479 Alpha-ketoisovaleric acid Alpha-ketoisovaleric acid is a branched chain organic acid which is a precursor to leucine and valine synthesis. It is also a degradation product from valine. The enzyme dihydroxy-acid dehydratase catalyzes the fourth step in the biosynthesis of isoleucine and valine, through the dehydration of 2, 3-dihydroxy-isovaleic acid into alpha-ketoisovaleric acid. 759-05-7 49 C5H8O3 White powder. 31.5°C No indication of carcinogenicity to humans (not listed by IARC). This is an endogenously produced metabolite found in the human body. It is used in metabolic reactions, catabolic reactions or waste generation. Chronically high levels of alpha-ketoisovaleric acid are associated with Maple Syrup Urine Disease. 2014-08-29T06:51:12Z 2026-04-02T23:14:53Z C00141 16530 2-KETO-ISOVALERATE DB04074 KIV true CC(C)C(=O)C(O)=O C5H8O3 InChI=1S/C5H8O3/c1-3(2)4(6)5(7)8/h3H,1-2H3,(H,7,8) QHKABHOOEWYVLI-UHFFFAOYSA-N 116.1152 116.047344122 Endogenous Solid HMDB00019 CHEMBL146554 48 CHEM003385 DTXSID6061078 3-methyl-2-oxobutanoic acid