GaA (Glucosidase Alpha, Acid, LYAG, Acid Alpha-Glucosidase, Lysosomal Alpha-Glucosidase, Pompe Disease Glycogen Storage Disease Type II, Acid Maltase, Aglucosidase Alfa)

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£495.00

SKU:: 363184
Anwendung:: WB
Reinheit:: Purified by Protein A and peptide affinity chromatography.
Lagerbedingungen:: -20°C
Hersteller:: United States Biological
Host:: Rabbit
Immunogen:: Recombinant protein corresponding to Glu70-Lys225 of rat GaA, expressed in E. coli.
Format:: Supplied as a liquid in 0.01M PBS, pH 7.4, 0.05% Proclin-300, 50% glycerol.
Weitere Details:: Lysosomal alpha-glucosidase is an enzyme that is encoded by the GAA gene. Error's in this gene cause glycogen storage disease type II (Pompe disease). 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 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. Other applications not tested. Recommended Dilution: 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