| CAS NO: | 759-05-7 |
| 包装 | 价格(元) |
| 100mg | 电议 |
| 500mg | 电议 |
| 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 |
| Canonical SMILES | CC(C(C(O)=O)=O)C |
| 运输条件 | 蓝冰运输或根据您的需求运输。 |
| 一般建议 | 为了使其更好的溶解，请用37℃加热试管并在超声波水浴中震动片刻。不同厂家不同批次产品溶解度各有差异，仅做参考。若实验所需浓度过大至产品溶解极限，请添加助溶剂助溶或自行调整浓度。溶液形式一般不宜长期储存，请尽快用完。 |
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.
m.cnreagent.com