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Isovaleryl-L-carnitine

品牌
Sigma-Aldrich
CAS
31023-24-2
货号
51371
规格纯度
≥94.0% (HPLC)
参考价格
738.32 *本价格含增值税费
促销
数量
-+
产品介绍:

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
Beilstein5946880
PubChem化学物质编号329757684
NACRESNA.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 stringC[N+](C)(C)C[C@H](OC(CC(C)C)=O)CC([O-])=O
InChI1S/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 keyIGQBPDJNUXPEMT-SNVBAGLBSA-N

安全信息

储存分类代码13 - Non Combustible Solids
WGKWGK 3
闪点(F)Not applicable
闪点(C)Not applicable

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