Target Synonym: GLA;GALA;Alpha-D-Galactoside;Galactohydrolase;galactosidase alpha;GLAL;Melibiase;Agalsidase;Alpha-galactosidase A;Alpha-D-galactosidase A;Alpha-D-galactoside galactohydrolase;Galactosylgalactosylglucosylceramidase GLA
Background: alpha -Galactosidase A is a homodimeric glycoprotein that can release terminal alpha -galactosyl moieties from glycolipids and glycoproteins and catalyze the hydrolysis of melibiose into galactose and glucose . It is a lysosomal enzyme and is responsible for degradation of glycolipid globotriaosylceramide (Gb3) (Gal alpha 1‑4Gal beta 1‑4Glc beta ‑ceramide). Mutations in this gene cause Fabry disease, an X-linked hereditary lysosomal storage disease with the accumulation of Gb3 in the walls of small blood vessels, nerves, dorsal root ganglia, renal glomerular and tubular epithelial cells, and cardiomyocytes .
Immunogen: Recombinant Human alpha-Galactosidase A/GLA protein
Buffer: 0.2 μm filtered solution in PBS
Dilution: WB 1:500-1:2000
Calculated MW: 49 kDa
Observed MW: 49 kDa
English
Deutsch
