Ketoisovaleric acid
Ketoisovaleric acid is an abnormal metabolite originated from the incomplete breakdown of branched-chain amino acids. Chronically high levels of ketoisovaleric acid are associated with maple syrup urine disease (MSUD). MSUD is an inherited metabolic disorder caused by deficiency of branched-chain α-keto acid dehydrogenase complex activity which leads to accumulation of the branched-chain α-keto acids including α-ketoisocaproic acid, α-ketoisovaleric acid and α-keto-β-methylvaleric acid, as well as their respective amino acids in blood and urine. From a chemistry perspective, ketoisovaleric acid is a keto acid, which is a subclass of organic acids. Abnormally high levels of organic acids in blood (organic acidemia), urine (organic aciduria), the brain, as well as other tissues result in general metabolic acidosis.
References:
1. HMDB0000019 (alpha-Ketoisovaleric acid). The Human Metabolome Database. Retrieved on September 7, 2021.
2. Bridi R, Braun CA, Zorzi GK, et al. alpha-keto acids accumulating in maple syrup urine disease stimulate lipid peroxidation and reduce antioxidant defences in cerebral cortex from young rats. Metabolic Brain Disease, 2005, 20(2): 155-167.
| Physical Appearance | A colorless liquid |
| Storage | Store at -20°C |
| M.Wt | 116.12 |
| Cas No. | 759-05-7 |
| Formula | C5H8O3 |
| Solubility | ≥11.6 mg/mL in DMSO; ≥27.05 mg/mL in H2O; ≥76.5 mg/mL in EtOH |
| Chemical Name | 3-methyl-2-oxobutanoic acid |
| SDF | Download SDF |
| Canonical SMILES | CC(C(C(O)=O)=O)C |
| Shipping Condition | Small Molecules with Blue Ice, Modified Nucleotides with Dry Ice. |
| General tips | We do not recommend long-term storage for the solution, please use it up soon. |
Quality Control & MSDS
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Chemical structure
