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GAA Antibody (N-term) Blocking peptide

Synthetic peptide

Product Information
Primary Accession P10253
Clone Names 80703075
Peptide ID 80703075
Additional Information
Gene ID 2548
Other Names Lysosomal alpha-glucosidase, Acid maltase, Aglucosidase alfa, 76 kDa lysosomal alpha-glucosidase, 70 kDa lysosomal alpha-glucosidase, GAA
Format Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.
StorageMaintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C.
PrecautionsThis product is for research use only. Not for use in diagnostic or therapeutic procedures.
Protein Information
Name GAA
Function Essential for the degradation of glycogen in lysosomes (PubMed:1856189, PubMed:7717400, PubMed:14695532, PubMed:18429042). Has highest activity on alpha-1,4-linked glycosidic linkages, but can also hydrolyze alpha-1,6-linked glucans (PubMed:29061980).
Cellular Location Lysosome. Lysosome membrane
Research Areas
Citations (0)

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This gene encodes acid alpha-glucosidase, which isessential for the degradation of glycogen to glucose in lysosomes.Different forms of acid alpha-glucosidase are obtained byproteolytic processing. Defects in this gene are the cause ofglycogen storage disease II, also known as Pompe's disease, whichis an autosomal recessive disorder with a broad clinical spectrum.Three transcript variants encoding the same protein have been foundfor 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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$ 80.00
Cat# BP12544a
Availability: In Stock
Bulk Size
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