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OG45939

Globotriaosylceramide

It is one of the few clusters of differentiation that is not a protein and structurally contains a galactose moiety linked α to lactosylceramide. Defects in the enzyme α-galactosidase lead to the buildup of globotriaosylceramide, causing Fabry's disease. The pharmaceutical drug migalastat enhances the function of α-galactosidase and is used to treat Fabry's.  Globotriaosylceramide is also one of the targets of Shiga toxin, which is responsible for pathogenicity of enterohemorrhagic E. coli.

Technical Data

CAS No: 71965-57-6
Synonyms: Gb3; a-D-Gal-(1→4)-b-D-Gal-(1→4)-b-D-Glc-1→O-ceramide; Ceramide trihexosides
Product Code: OG45939
Chemical Formula: C60H113NO18
Molecular Weight: 1136.54

References


  • Desnick RJ and Ioannou YA (2001). α-Galactosidase A deficiency: Fabry disease. In: Scriver, R., et al., editors. The Metabolic and Molecular Bases of Inherited Disease. New York: McGraw-Hill, 3733–3774.
  • Sweeley CC and Klionsky B (1963). Fabry’s Disease: Classification as a Sphingolipidosis and Partial Characterization of a Novel Glycolipid. Journal of Biological Chemistry 238(9): 3148.

Solubility

Stability

Further Information


Datasheets

Specification: View
MSDS: View

CAS No:
71965-57-6
Synonyms:
Gb3
a-D-Gal-(1→4)-b-D-Gal-(1→4)-b-D-Glc-1→O-ceramide
Ceramide trihexosides
Chemical Formula:
Molecular Weight:
1136.54
Product Structure
References:
1. Desnick RJ and Ioannou YA (2001). α-Galactosidase A deficiency: Fabry disease. In: Scriver, R., et al., editors. The Metabolic and Molecular Bases of Inherited Disease. New York: McGraw-Hill, 3733–3774.
2. Sweeley CC and Klionsky B (1963). Fabry’s Disease: Classification as a Sphingolipidosis and Partial Characterization of a Novel Glycolipid. Journal of Biological Chemistry 238(9): 3148.
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