3-Methyl-2-oxobutanoic acid - CAS 759-05-7

3-Methyl-2-oxobutanoic acid (2-Oxoisovaleric acid) is an abnormal metabolite that arises from the incomplete breakdown of branched-chain amino acids. 3-Methyl-2-oxobutanoic acid is a neurotoxin, an acidogen, and a metabotoxin. A neurotoxin causes damage to nerve cells and nerve tissues. 3-Methyl-2-oxobutanoic acid is a keto-acid, which is a subclass of organic acids. Abnormally high levels of organic acids in the blood (organic acidemia), urine (organic aciduria), the brain, and other tissues lead to general metabolic acidosis.

Endogenous Metabolite

Metabolism

759-05-7

0.98

C5H8O3

116.12

CC(C)C(=O)C(O)=O

-20°C

Ice Packs

Natural Product