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Lyso-Globotriaosylceramide (d18:1)

(Synonyms: Globotriaosylsphingosine (d18:1), Lyso-Ceramide Trihexoside, Lyso-Gb3) 目录号 : GC44101

A globotriaosylceramide lacking the fatty acyl group

Lyso-Globotriaosylceramide (d18:1) Chemical Structure

Cas No.:126550-86-5

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1mg
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产品描述

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.

Chemical Properties

Cas No. 126550-86-5 SDF
别名 Globotriaosylsphingosine (d18:1), Lyso-Ceramide Trihexoside, Lyso-Gb3
Canonical SMILES O[C@H]1[C@H](O[C@]2([H])O[C@H](CO)[C@H](O[C@]3([H])[C@H](O)[C@@H](O)[C@@H](O)[C@@H](CO)O3)[C@H](O)[C@H]2O)[C@@H](CO)O[C@@H](OC[C@H](N)[C@H](O)/C=C/CCCCCCCCCCCCC)[C@@H]1O
分子式 C36H67NO17 分子量 785.9
溶解度 Chloroform:Methanol:Water (4:3:1): Soluble 储存条件 Store at -20°C
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1 mg 5 mg 10 mg
1 mM 1.2724 mL 6.3621 mL 12.7243 mL
5 mM 0.2545 mL 1.2724 mL 2.5449 mL
10 mM 0.1272 mL 0.6362 mL 1.2724 mL
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