Publisher Summary Glycosphingolipids are ubiquitous but poorly understood membrane
components. They amass because of defective glycosidases or activator proteins for these
glycosidases in several sphingolipid storage disorders. These disorders include Gaucher,
Fabry, and Tay-Sachs disease where glucosylceramide, globotriaosylceramide (Gb3), and
ganglioside GM2, respectively, accumulate within the affected tissues. Glucosylceramide is
the base cerebroside for the majority of glycosphingolipids, including Gb3 and ganglioside …