Metabolites

3-Methyl-2-oxobutanoic acid View larger

3-Methyl-2-oxobutanoic acid

AT5232

CAS 759-05-7

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Data sheet

Molecular FormulaC5H8O3
Molecular Weight116.12
CAS Numbers759-05-7
Storage Condition0C Short Term, -20C Long Term
SolubilityDMSO
Purity98% by HPLC
SMILES CodeCC(C)C(=O)C(O)=O
ReferencesYudkoff M, et al. Brain amino acid requirements and toxicity the example of leucine. J Nutr. 2005 Jun;135[6 Suppl] 1531S-8S.

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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.