Background: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.
仕様
Synonyms:AGAL,Agalsidase alfa,Alpha D galactosidase A,Alpha D galactoside galactohydrolase 1,Alpha D galactoside galactohydrolase,Alpha gal A,Alpha galactosidase A,Alpha-D-galactosidase A,Alpha-D-galactoside galactohydrolase,Alpha-galactosidase A,GALA,Galactosidase,alpha,GLA,GLA protein,Melibiase
Host:Rabbit
Reactivity:Human
Applications:WB
Concentration:1mg/ml
Immunogen:Recombinant protein of human GLA
Purification Method:Affinity purification
Clonality:Polyclonal
Conjugation:Unconjugated
Buffer:Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.