Species: | Rabbit |
Applications: | WB IHC IF |
Immunogen Range: | Recombinant protein of human GLA |
Clonality: | Polyclonal Antibody |
Isotype: | IgG |
GENE ID: | 2717 |
Swiss Prot: | P06280 |
Synonyms: | GALA |
Purification: | Affinity purification |
Storage: | Store at -20°C (regular) or -80°C (long term). Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
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. |
Caculated MW: | 49 kDa |
Observed MW: | Refer to Figures |
Applications: |
WB 1:500-1:2000 IHC 1:50-1:200 IF 1:50-1:100 |
Reacitivity: | Human, Mouse, Rat |