Formulation: A solid
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
Purity: ≥98%
Formula Markup: C36H67NO17
Formula Weight: 785,9
Shelf life (days): 1460
CAS Number: 126550-86-5
Notes: 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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