Globotriaosylceramide
Globotriaosylceramide is a ganglioside.[1]
It is also known as CD77, Gb3, and ceramide trihexoside.[2] It is one of the few clusters of differentiation that is not a protein.
It is formed by the alpha linkage of galactose to lactosylceramide catalyzed by A4GALT.
It is metabolized by alpha-galactosidase, which hydrolyzes the terminal alpha linkage.
Clinical significance
Defects in the enzyme alpha-galactosidase lead to the build up of globotriaosylceramide, causing Fabry's disease.[3]
Globotriaosylceramide is also one of the targets of Shiga toxin, which is responsible for pathogenicity of enterohemorrhagic Escherichia coli (EHEC). Additionally, many studies have found that globotriaosylceramide is the primary receptor for the Cholera toxin, the main mechanism by which Vibrio cholerae infections elicits severe diarrhea.
References
- ↑ globotriaosylceramide at the US National Library of Medicine Medical Subject Headings (MeSH)
- ↑ Bekri S, Lidove O, Jaussaud R, Knebelmann B, Barbey F (October 2006). "The role of ceramide trihexoside (globotriaosylceramide) in the diagnosis and follow-up of the efficacy of treatment of Fabry disease: a review of the literature". Cardiovasc Hematol Agents Med Chem 4 (4): 289–97. doi:10.2174/187152506778520718. PMID 17073606.
- ↑ Desnick RJ, Ioannou YA, Eng CM. a-Galactosidase A deficiency: Fabry disease. In: Scriver CR, Beaudet AL, Sly WS, Valle D, eds. The metabolic & molecular bases of inherited disease. 8th ed. Vol. 3. New York: McGraw-Hill, 2001:3733-74.
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