Lyso-Globotriaosylceramide (d18:1)

A globotriaosylceramide lacking the fatty acyl group

此产品仅用于科学研究，我们不为任何个人用途提供产品和服务

库存: 现货

可选规格

包装

价格

促销价

数量
1mg

￥13187.00

10550.00

- +

已选 0 种 0 瓶

金额: ￥0.00

首页收藏

货号: ajci72444
CAS: 126550-86-5
别名: Globotriaosylsphingosine (d18:1), Lyso-Ceramide Trihexoside, Lyso-Gb3
分子式: C36H67NO17
分子量: 785.9
纯度: >98%
溶解度: Chloroform:Methanol:Water (4:3:1): Soluble
储存: Store at -20°C
库存: 现货

Background

Lyso-globotriaosylceramide is a form of globotriaosylceramide that is lacking the fatty acyl group. It binds to Shiga toxin 1 (Stx1) in the presence of cholesterol and phosphatidylcholine but does not bind Stx2. It also reduces viability and aggregation of human neutrophils induced by phorbol 12-myristate 13-acetate when used at concentrations of 50 and 1 μM, respectively. Lyso-globotriaosylceramide accumulates in the brain, heart, kidney, liver, lung, and spleen in a mouse model of Fabry disease, a lysosomal storage disorder characterized by a deficiency in the enzyme α-galactosidase A. It also accumulates in the urine, kidney, and plasma of patients with Fabry disease. Lyso-globotriaosylceramide levels decrease in response to administration of the α-galactosidase inhibitor 1-deoxygalactonojirimycin in a transgenic mouse model of Fabry disease. Decreases in plasma and urine concentrations of lyso-globotriaosylceramide have been used as a biomarker for efficacy of enzyme replacement therapy (ERT) and other therapies in the treatment of Fabry disease.