Anti-GAA, NT (GAA, Lysosomal alpha-glucosidase, Acid maltase, Aglucosidase alfa, 76kD lysosomal alph

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. Applications: Suitable for use in Western Blot, Immunohistochemistry and ELISA. Other applications have not been tested. Recommended Dilutions: Western Blot: 1:1000, Immunohistochemistry (Fozen): 1:25, Immunohistochemistry (FFPE): 1:10-1:50, Optimal dilutions to be determined by the researcher. Storage and Stability: May be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Aliquots are stable for 12 months after receipt. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.