Alpha galactosidase A Polyclonal Antibody

Rs. 21,832.00
SKU E-AB-64886


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
Swissprot P06280
Source Rabbit
Reactivity Human
Immunogen Recombinant protein of human GLA
Application WB
Recommended dilution WB 1:500 - 1:2000
Concentration 1mg/ml
Clonality Polyclonal


Cellular localization  
Tissue specificity  
Isotype IgG
Purification Affinity purification
Conjugation Unconjugated
Storage instructions Store at -20℃. Avoid freeze / thaw cycles.
Storage buffer 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.


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