|Application ||WB, IHC-P, IF, FC, E|
|Calculated MW||48767 Da|
|Antigen Region||83-112 aa|
|Other Names||Alpha-galactosidase A, Alpha-D-galactosidase A, Alpha-D-galactoside galactohydrolase, Melibiase, Agalsidase, GLA|
|Target/Specificity||This GLA antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 83-112 amino acids from the N-terminal region of human GLA.|
|Format||Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is prepared by Saturated Ammonium Sulfate (SAS) precipitation followed by dialysis against PBS.|
|Storage||Maintain refrigerated at 2-8°C for up to 2 weeks. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles.|
|Precautions||GLA Antibody (N-term) is for research use only and not for use in diagnostic or therapeutic procedures.|
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Provided below are standard protocols that you may find useful for product applications.
GLA is 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.
Mignani,R., Kidney Int. 75 (10), 1115-1116 (2009)
Ioannou,Y.A., Biochem. J. 332 (PT 3), 789-797 (1998)
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