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

Produktübersicht

• Artikelname: Lyso-Globotriaosylceramide (d18:1), CAS [[126550-86-5]]
• Artikelnummer: TGM-T37291
• Hersteller Artikelnummer: T37291
• Alternativnummer: TGM-T37291-1MG
• Hersteller: TargetMol
• Kategorie: Biochemikalien
• Alternative Synonym: Lyso-Globotriaosylceramide (d18:1)

Produktbeschreibung

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.

Produkteigenschaften

• Molekulargewicht: 785.922
• CAS Nummer: [126550-86-5]
• Formel: C36H67NO17
• Target-Kategorie: Calcium Channel|||Others

Bilder

T37291