3-Methyl-2-oxobutanoic acid

Alpha-Ketoisovaleric acid is an abnormal metabolite that arises from the incomplete breakdown of branched-chain amino acids. alpha-Ketoisovaleric acid is a neurotoxin an acidogen and a metabotoxin. A neurotoxin causes damage to nerve cells and nerve tissues. alpha-Ketoisovaleric 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.