Globotriaosylceramide is a ganglioside. [1 ]
It is also known as CD77, Gb3, and ceramide trihexoside.
It is one of the few [2 ] 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 [ edit ]
Defects in the enzyme
alpha-galactosidase lead to the build up of globotriaosylceramide, and this is the cause of Fabry's disease. [3 ]
Globotriaosylceramide is also the one of the targets of
Shiga toxin, which is responsible for pathogenicity of enterohemorrhagic Escherichia coli (EHEC).
References [ edit ]
^ 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.