||Recombinant human GAA protein was expressed in HEK293T.
||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.
||Lyophilized from sterile PBS (pH7.4), 300 mM NaCl, 1 mM DTT.
||Hydrolysis of terminal, non-reducing (1->4)-linked alpha-D-glucose residues with release of alpha-D-glucose.
||The recombinant α-glucosidase comprises 952 amino acids with a predicted MW of 105 kDa. Then it is cleaved into the following 2 chains: 76 kDa or 70 kDa during post-translational modification.
||Store it at +4ºC for short term (4 weeks). For long term storage( 12 months), store it at -20ºC~-70ºC from date of receipt. Avoid freeze-thaw cycles.
||It is recommended that sterile water be added to the vial to prepare a stock solution of 0.2 ug/ul. Centrifuge the vial at 4ºC before opening to recover the entire contents.