α-galactosidase A

a lysosomal enzyme that catalyzes the cleavage of terminal galactose residues from glycosphingolipids, particularly ceramide trihexosides. Deficiency of the enzyme, an X-linked trait, causes accumulation of ceramide trihexosides and other glycosphingolipids in plasma and tissues and results in Fabry disease. Called also ceramide trihexosidase and α-g.