|Application ||WB, IHC, IHC-P, E|
|Other Accession||NP_000143.2, NP_001073271.1|
|Calculated MW||105324 Da|
|Antigen Region||174-203 aa|
|Other Names||Lysosomal alpha-glucosidase, Acid maltase, Aglucosidase alfa, 76 kDa lysosomal alpha-glucosidase, 70 kDa lysosomal alpha-glucosidase, GAA|
|Target/Specificity||This GAA antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 174-203 amino acids from the N-terminal region of human GAA.|
|Format||Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.|
|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||GAA Antibody (N-term) is for research use only and not for use in diagnostic or therapeutic procedures.|
|Function||Essential for the degradation of glygogen to glucose in lysosomes.|
|Cellular Location||Lysosome. Lysosome membrane|
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Provided below are standard protocols that you may find useful for product applications.
This gene encodes acid alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. Different forms of acid alpha-glucosidase are obtained by proteolytic processing. Defects in this gene are the cause of glycogen storage disease II, also known as Pompe's disease, which is an autosomal recessive disorder with a broad clinical spectrum. Three transcript variants encoding the same protein have been found for this gene.
Bailey, S.D., et al. Diabetes Care 33(10):2250-2253(2010)
Labrousse, P., et al. Mol. Genet. Metab. 99(4):379-383(2010)
Talmud, P.J., et al. Am. J. Hum. Genet. 85(5):628-642(2009)
Aoyama, Y., et al. J. Hum. Genet. 54(11):681-686(2009)
Maimaiti, M., et al. J. Hum. Genet. 54(8):493-496(2009)
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