产品说明
生化/生理作用
Isovalerylcarnitine is the phenotypic abnormality in iso-valeric acidemia resulting from an accumulation of isovaleric acid, which is toxic to the central nervous system. Isovaleric acidemia is an autosomal recessive inborn error of leucine metabolism caused by a deficiency of the mitochondrial enzyme isovaleryl-CoA dehydrogenase resulting in the accumulation of derivatives of isovaleryl-CoA. It was the first organic acidemia recognized in humans and can cause significant morbidity and mortality. Early diagnosis and treatment with a protein restricted diet and supplementation with carnitine and glycine are effective in promoting normal development in severely affected individuals. An alternative pathway through glycine-N-acylase allows detoxification by producing isovaleryl-glycine, which is excreted. Thus, isovalerylcarnitine and isovalerylglycine are the hallmarks of this disorder in plasma and urine, respectively, and are elevated regardless of a patient′s metabolic condition.
基本信息
| 经验(实验)分子式 | C12H23NO4 |
| 分子量 | 245.32 |
| Beilstein | 5946880 |
| PubChem化学物质编号 | 329757684 |
| NACRES | NA.26 |
产品性质
| 质量水平 | 100 |
| 测定 | ≥94.0% (HPLC) |
| 形式 | powder or crystals |
| 旋光性 | [α]/D -23±2°, c = 1 in H2O |
| 杂质 | ≤10% water |
| 颜色 | white to off-white |
| 储存温度 | 2-8℃ |
| SMILES string | C[N+](C)(C)C[C@H](OC(CC(C)C)=O)CC([O-])=O |
| InChI | 1S/C12H23NO4/c1-9(2)6-12(16)17-10(7-11(14)15)8-13(3,4)5/h9-10H,6-8H2,1-5H3/t10-/m1/s1 |
| InChI key | IGQBPDJNUXPEMT-SNVBAGLBSA-N |
安全信息
| 储存分类代码 | 13 - Non Combustible Solids |
| WGK | WGK 3 |
| 闪点(F) | Not applicable |
| 闪点(C) | Not applicable |
Sigma-Aldrich
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