A globotriaosylceramide lacking the fatty acyl group; binds to Stx1 in the presence of cholesterol and phosphatidylcholine

Lyso-Globotriaosylceramide (d18:1) – 1 mg

Brand:
Cayman
CAS:
126550-86-5
Storage:
-20
UN-No:
Non-Hazardous - /

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.{36643} It also reduces viability and aggregation of human neutrophils induced by phorbol 12-myristate 13-acetate (PMA; Item No. 10008014) when used at concentrations of 50 and 1 μM, respectively.{38972} 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.{41495} It also accumulates in the urine, kidney, and plasma of patients with Fabry disease.{36644} Lyso-globotriaosylceramide levels decrease in response to administration of the α-galactosidase inhibitor 1-deoxygalactonojirimycin (migalastat; Item No. 17179) 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. [Matreya, LLC. Catalog No. 1520]  

 

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Description

A globotriaosylceramide lacking the fatty acyl group; binds to Stx1 in the presence of cholesterol and phosphatidylcholine, but does not bind Stx2; reduces viability and PMA-induced aggregation of human neutrophils (50 and 1 μM, respectively); accumulates in various organs in a mouse model of Fabry disease; accumulates in urine, kidneys, and plasma of patients with Fabry disease; decreases in response to administration of 1-deoxygalactonojirimycin in a transgenic mouse model of Fabry disease; has been used as a biomarker for efficacy of ERT and other therapies in the treatment of Fabry disease,

Formal name: (2S,3R,4E)-2-amino-3-hydroxy-4-octadecen-1-yl O-α-D-galactopyranosyl-(1→4)-O-β-D-galactopyranosyl-(1→4)-β-D-glucopyranoside

Synonyms:  Globotriaosylsphingosine (d18:1)|Lyso-Ceramide Trihexoside|Lyso-Gb3

Molecular weight: 785.9

CAS: 126550-86-5

Purity: ≥98%

Formulation: A solid

Product Type|Biochemicals|Lipids|Sphingolipids||Research Area|Endocrinology & Metabolism|Inborn Errors of Metabolism||Research Area|Immunology & Inflammation|Innate Immunity||Research Area|Infectious Disease|Bacterial Diseases|Shigellosis||Research Area|Lipid Biochemistry|Sphingolipids

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