363185
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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£581.00
- SKU:
- 363185
- Reinheit:
- Purified by immunoaffinity chromatography.
- Lagerbedingungen:
- -20°C
- Hersteller:
- United States Biological
- Host:
- Rabbit
- Immunogen:
- Recombinant protein corresponding to Glu70-Phe245 of mouse GaA, expressed in E. coli.
- Format:
- Supplied as a liquid in PBS, pH 7.4, 0.02% sodium azide, 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 ELISA, Western Blot, Immunohistochemistry, Immunocytochemistry. Other applications not tested. Recommended Dilution: ELISA: 1:100-1:5000 Western Blot: 1:50-400 Immunohistochemistry (frozen): 1:50-500 Immunohistochemistry (paraffin): 1:10-100 Immunocytochemistry: 1:50-500 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