Globotriaosylceramide
Globotriaosylceramide is a globoside.[1] It is also known as CD77, Gb3, GL3, 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
Clinical significance
Defects in the enzyme
Globotriaosylceramide is also one of the targets of Shiga toxin, which is responsible for pathogenicity of enterohemorrhagic Escherichia coli (EHEC).[citation needed]
The bacterial Shiga toxin can be used for targeted therapy of certain gastrointestinal cancers that express the receptor of the Shiga toxin.[4] For this purpose a non-specific chemotherapeutic agent is conjugated to the B-subunit to make it specific. In this way only the tumor cells, but not healthy cells, should be destroyed during therapy.[5]
References
- ^ globotriaosylceramide at the U.S. National Library of Medicine Medical Subject Headings (MeSH)
- PMID 17073606. Archived from the originalon 2013-04-14.
- ^ 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.
- PMC 2730034. e6813.
- .
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