Globotriaosylceramide (Gb3)

Globotriaosylceramide (Gb3), also known as CD77, GL3, and ceramide trihexoside, is a membrane glycosphingolipid crucially involved both physiologically—in cell signaling—and pathologically—as substrate accumulating due to enzyme deficiency or acting as microbial/toxin receptor—with major implications especially for Fabry disease diagnosis/therapy and certain cancer-targeted treatments. It consists of a ceramide backbone linked to an oligosaccharide chain composed of two galactose units and one glucose unit. Gb3 acts as a receptor for Shiga toxin and accumulates in Fabry disease due to a deficiency in alpha-galactosidase A. It is also a target for cancer therapy.

Other names

CD77GL3Ceramide trihexosideCer-Trihex

Migalastat enhances residual activity of mutant forms of alpha-galactosidase A. Modified Shiga toxin subunits deliver cytotoxic agents specifically against tumor cells expressing Gb3.