For research use only. Not for therapeutic Use.
3-Methyl-2-oxobutanoic acid（Cat No.:I019916）, also known as alpha-Ketoisovalerate or alpha-Ketoisovaleric acid, is a neurotoxin that is produced as an abnormal metabolite during the incomplete breakdown of branched-chain amino acids. It is a byproduct of amino acid metabolism and can accumulate in certain metabolic disorders, such as maple syrup urine disease. The accumulation of alpha-Ketoisovalerate can lead to neurotoxic effects, causing damage to the nervous system.
| Catalog Number | I019916 |
| CAS Number | 759-05-7 |
| Molecular Formula | C₅H₈O₃ |
| Purity | ≥95% |
| Target | Endogenous Metabolite |
| Storage | 2-8°C |
| IUPAC Name | 3-methyl-2-oxobutanoic acid |
| InChI | InChI=1S/C5H8O3/c1-3(2)4(6)5(7)8/h3H,1-2H3,(H,7,8) |
| InChIKey | QHKABHOOEWYVLI-UHFFFAOYSA-N |
| SMILES | CC(C)C(=O)C(=O)O |
| Reference | [1]. MAAS WK, et al. alpha-Ketoisovaleric acid, a precursor of pantothenic acid in Escherichia coli. J Bacteriol. 1953 Apr;65(4):388-93.<br>[2]. Schauder P, et al. Oral administration of alpha-ketoisovaleric acid or valine in humans: blood kinetics and biochemical effects. J Lab Clin Med. 1984 Apr;103(4):597-605.<br>[3]. Coitinho AS, et al. Pharmacological evidence that alpha-ketoisovaleric acid induces convulsions through GABAergic and glutamatergic mechanisms in rats. Brain Res. 2001 Mar 9;894(1):68-73. |
