Lyso-Globotriaosylceramide (d18:1), CAS [[126550-86-5]]

Product Overview

• Article Name: Lyso-Globotriaosylceramide (d18:1), CAS [[126550-86-5]]
• Biozol Catalog Number: TGM-T37291
• Supplier Catalog Number: T37291
• Alternative Catalog Number: TGM-T37291-1MG
• Manufacturer: TargetMol
• Category: Biochemikalien
• Alternative Names: Lyso-Globotriaosylceramide (d18:1)

Product Description

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 alpha-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 alpha-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.

Product Properties

• Molecular Weight: 785.922
• CAS Number: [126550-86-5]
• Formula: C36H67NO17
• Target: Calcium Channel|||Others

Images

T37291