481469
GAA (Lysosomal alpha-Glucosidase, Glucosidase, alpha; Acid)
Size
£614.00
- SKU:
- 481469
- Purity:
- Purified by affinity chromatography.
- Storage Conditions:
- -20°C
- Supplier:
- 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.
- Extra 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.
- Shipping Conditions:
- Blue Ice