Galactosidase alpha Mouse Monoclonal Antibody [A7C4] (HA600088)

UNIPROT #

P06280

PROTEIN NAME

Galactosidase alpha

SYNONYMS

AGAL_HUMAN antibody;Agalsidase alfa antibody;Alpha D galactosidase A antibody;Alpha D galactoside galactohydrolase 1 antibody;Alpha D galactoside galactohydrolase antibody;Alpha gal A antibody;Alpha galactosidase A antibody;Alpha-D-galactosidase A antibody;Alpha-D-galactoside galactohydrolase antibody;Alpha-galactosidase A antibody;GALA antibody;Galactosidase, alpha antibody;GLA antibody;GLA protein antibody;Melibiase antibody

SUBCELLULAR LOCATION

Lysosome.

FUNCTION

Alpha-galactosidase (α-GAL, also known as α-GAL A; E.C. 3.2.1.22) is a glycoside hydrolase enzyme that hydrolyses the terminal alpha-galactosyl moieties from glycolipids and glycoproteins. Glycosidase is an important class of enzyme catalyzing many catabolic processes, including cleaving glycoproteins and glycolipids, and polysaccharides. Specifically, α-GAL catalyzes the removal of the terminal α-galactose from oligosaccharides. The enzyme is encoded by the GLA gene. Two recombinant forms of human alpha-galactosidase are called agalsidase alpha (INN) and agalsidase beta (INN). A mold-derived form is the primary ingredient in gas relief supplements. This enzyme is a homodimeric glycoprotein that hydrolyses the terminal alpha-galactosyl moieties from glycolipids and glycoproteins. It predominantly hydrolyzes ceramide trihexoside, and it can catalyze the hydrolysis of melibiose into galactose and glucose.