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Product Name | Lyso-Globotriaosylceramide (d18:1) |
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, |
Size | 1 mg |
Concentration | n/a |
Applications | n/a |
Other Names | Globotriaosylsphingosine (d18:1)|Lyso-Ceramide Trihexoside|Lyso-Gb3 |
Gene, Accession, CAS # | CAS: 126550-86-5 |
Catalog # | 24873-1 |
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Order / More Info | Lyso-Globotriaosylceramide (d18:1) from CAYMAN CHEMICAL COMPANY |
Product Specific References | n/a |
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