GAA (Lysosomal alpha-Glucosidase, Glucosidase, alpha; Acid)

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£614.00

SKU:: 481469
Reinheit:: Purified by affinity chromatography.
Lagerbedingungen:: -20°C
Hersteller:: United States Biological
Host:: Rabbit
Immunogen:: Synthetic peptide corresponding to the N-terminal region of human GAA
Format:: Supplied as a liquid in PBS, 2% sucrose, 0.09% sodium azide.
Weitere Details:: GAA is 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. 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 Immunohistochemistry, 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.

Versandbedingungen:: Blue Ice