异戊酰-L-肉碱

别名 (2 R )-3-羧基- N , N , N -三甲基-2-(3-甲基-1-氧代丁氧基)-1-丙胺 内盐|L-肉碱异戊酸酯|iC5-肉碱|
英文别名 Isovalerylcarnitine|3-Methylbutyrylcarnitine|31023-24-2|O-isovalerylcarnitine|3-[(3-methylbutanoyl)oxy]-4-(trimethylazaniumyl)butanoate|Isovaleryl L-carnitine|1-Propanaminium, 3-carboxy-N,N,N-trimethyl-2-(3-methyl-1-oxobutoxy)-, inner salt, (2R)-|ISOVALER
规格或纯度 ≥94.0% (HPLC)
英文名称 Isovaleryl-L-carnitine 生化机理 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.
储存温度 2-8°C储存,充氩
运输条件 冰袋运输
产品介绍
产品介绍： Isovalerylcarnitine 是 L-亮氨酸分解代谢的产物。Isovalerylcarnitine 也是一种具有选择性和可逆性的钙蛋白酶 (calpain) 激活剂，可诱导细胞凋亡。 Product description： Isovalerylcarnitine is a product of the catabolism of L-leucine. Isovalerylcarnitine is also a selective and reversible calpain activator that induces apoptosis.