|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.|
|Storage||Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C.|
|Precautions||This product is for research use only. 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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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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