Galactosidase Alpha; Alpha-D-Galactosidase A; EC 3.2.1.22; Melibiase; Alpha-D-Galactoside Galactohydrolase 1; Alpha-D-Galactoside Galactohydrolase; Alpha-Galactosidase A

This gene encodes a homodimeric glycoprotein that hydrolyses the terminal alpha-galactosyl moieties from glycolipids and glycoproteins. This enzyme predominantly hydrolyzes ceramide trihexoside, and it can catalyze the hydrolysis of melibiose into galactose and glucose. A variety of mutations in this gene affect the synthesis, processing, and stability of this enzyme, which causes Fabry disease, a rare lysosomal storage disorder that results from a failure to catabolize alpha-D-galactosyl glycolipid moieties.