Galactosidase alpha (GLA) Rabbit pAb (20 μl)

Galactosidase alpha (GLA) Rabbit pAb (20 μl)

Brand: Abclonal
Cat. #: A13987
Availability: In Stock
$108.00
-+
Reactivity: Human    
Applications: WB, ELISA    
Host Species: Rabbit    
Isotype: IgG    
Clonality: Polyclonal antibody    
Gene Name: galactosidase alpha
Gene Symbol: GLA
Synonyms: GALA; Galactosidase alpha (GLA)
Gene ID: 2717
UniProt ID: P06280
Immunogen: Recombinant fusion protein containing a sequence corresponding to amino acids 150-429 of human Galactosidase alpha (Galactosidase alpha (GLA)) (NP_000160.1).
Dilution: WB 1:500-1:2000
Purification Method: Affinity purification
Concentration: 0.96 mg/ml
Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Storage: Store at -20°C. Avoid freeze/thaw cycles.
Documents: Manual-GLA antibody




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.




Images

DUSP16 antibodyWestern blot analysis of lysates from HeLa cells, using Galactosidase alpha (GLA) Rabbit pAb (A13987) at 1:1000 dilution.
Secondary antibody: HRP-conjugated Goat anti-Rabbit IgG (H+L) (AS014) at 1:10000 dilution.
Lysates/proteins: 25μg per lane.
Blocking buffer: 3% nonfat dry milk in TBST.
Detection: ECL Basic Kit (RM00020).
Exposure time: 30s.



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