靶标:
GLA
产品别名:
GALA; GLA; galactosidase alpha; galactosidase alpha; alpha-galactosidase A; agalsidase alfa; alpha-D-galactosidase A; alpha-D-galactoside galactohydrolase 1; alpha-gal A; galactosylgalactosylglucosylceramidase GLA; melibiase; α-半乳糖苷酶; 半乳糖苷酶α(GLa);
背景信息:
Alpha-galactosidase is a glycoside hydrolase enzyme that encoded by the GLA gene. This gene is 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.