|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|
Thousands of laboratories across the world have published research that depended on the performance of antibodies from Abgent to advance their research. Check out links to articles that cite our products in major peer-reviewed journals, organized by research category.
email@example.com, and receive a free "I Love Antibodies" mug.
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)
If you have used an Abgent product and would like to share how it has performed, please click on the "Submit Review" button and provide the requested information. Our staff will examine and post your review and contact you if needed.
If you have any additional inquiries please email technical services at firstname.lastname@example.org.