It is formed by the alpha linkage of galactose to lactosylceramide catalyzed by A4GALT.
Defects in the enzyme alpha-galactosidase lead to the buildup of globotriaosylceramide, causing Fabry's disease.
[3] The pharmaceutical drug migalastat enhances the function of alpha-galactosidase and is used to treat Fabry's.
[citation needed] Globotriaosylceramide is also one of the targets of Shiga toxin, which is responsible for pathogenicity of enterohemorrhagic Escherichia coli (EHEC).
[4] For this purpose a non-specific chemotherapeutic agent is conjugated to the B-subunit to make it specific.