Alpha-galactosidase (GALA) is a glycoside hydrolase enzyme that hydrolyses the terminal alpha-galactosyl moieties from glycolipids and glycoproteins. It is encoded by the GLA gene. This enzyme predominantly hydrolyzes ceramide trihexoside, and it can catalyze the hydrolysis of melibiose into galactose and glucose. Inherited mutations in the gene encoding α-gal A cause an X-linked recessive glycolipid storage disorder known as Fabry’s disease. In Fabry patients, α-gal A deficiencies lead to an accumulation of Gb3 in the body. Enzyme replacement therapy using recombinant α-gal A effectively treats the symptoms of Fabry disease.
