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Lyso-Globotriaosylceramide (d18:1)

Lyso-Globotriaosylceramide (d18:1)

产品编号 T37291   CAS 126550-86-5
别名: Lyso-Globotriaosylceramide (d18:1)

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.

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Lyso-Globotriaosylceramide (d18:1) Chemical Structure
Lyso-Globotriaosylceramide (d18:1), CAS 126550-86-5
规格 价格/CNY 货期 数量
1 mg ¥ 11,700 35日内发货
产品目录号及名称: Lyso-Globotriaosylceramide (d18:1) (T37291)
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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.
别名 Lyso-Globotriaosylceramide (d18:1)
分子量 785.922
分子式 C36H67NO17
CAS No. 126550-86-5

存储

Powder: -20°C for 3 years | In solvent: -80°C for 1 year

溶解度

Chloroform:Methanol:H2O (4:3:1): Soluble

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Keywords

Lyso-Globotriaosylceramide (d18:1) 126550-86-5 LysoGlobotriaosylceramide (d18:1) Lyso Globotriaosylceramide (d18:1) Lyso-Globotriaosylceramide Lyso-Globotriaosylceramide (d-18:1) d18:1 Inhibitor inhibitor inhibit

 

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