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Lyso-Globotriaosylceramide (d18:1)

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    Lyso-Globotriaosyl<wbr/>ceramide (d18:1)
  • Lyso-Globotriaosyl<wbr/>ceramide (d18:1)
Cat No: 24873
Biochemicals - Lipids
Cayman

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 ag...

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: 1 mg

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Territorial Availability: Available through Bertin Technologies only in France
Synonyms:
  • (2S,3R,4E)-2-amino-3-hydroxy-4-octadecen-1-yl O-?-D-galactopyranosyl-(1?4)-O-?-D-galactopyranosyl-(1?4)-?-D-glucopyranoside
Correlated keywords:
  • 1581768-73-1 globotriaosyl-ceramide sphingosine Stx-1 2 deoxy-galactonojirimycin
Product Overview:
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]
Size 1 mg
Shipping dry ice
CAS Number 126550-86-5
Molecular Formula C36H67NO17
SMILES O[C@H]1[C@H](O[C@]2([H])O[C@H](CO)[C@H](O[C@]3([H])[C@H](O)[C@@H](O)[C@@H](O)[C@@H](CO)O3)[C@H](O)[C@H]2O)[C@@H](CO)O[C@@H](OC[C@H](N)[C@H](O)/C=C/CCCCCCCCCCCCC)[C@@H]1O
Molecular Weight 785,9
Formulation A solid
Purity ≥98%
Custom Code 2921.19
UNSPSC code 12352100

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