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globotriaosylceramide : ウィキペディア英語版
globotriaosylceramide
Globotriaosylceramide is a ganglioside.
It is also known as CD77, Gb3, and ceramide trihexoside. 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.〔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.〕
Globotriaosylceramide is also one of the targets of Shiga toxin, which is responsible for pathogenicity of enterohemorrhagic Escherichia coli (EHEC).

抄文引用元・出典: フリー百科事典『 ウィキペディア(Wikipedia)
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