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Metabolites
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View larger 3-Methyl-2-oxobutanoic acid
AT5232
CAS 759-05-7
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Data sheet
| Molecular Formula | C5H8O3 |
| Molecular Weight | 116.12 |
| CAS Numbers | 759-05-7 |
| Storage Condition | 0C Short Term, -20C Long Term |
| Solubility | DMSO |
| Purity | 98% by HPLC |
| SMILES Code | CC(C)C(=O)C(O)=O |
| References | Yudkoff M, et al. Brain amino acid requirements and toxicity the example of leucine. J Nutr. 2005 Jun;135[6 Suppl] 1531S-8S. |
More info
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.