Globotriaosylceramide

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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[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]

  1. ^ globotriaosylceramide at the US National Library of Medicine Medical Subject Headings (MeSH)
  2. ^ 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. 
  3. ^ 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.