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: 1:100-1:5000Western Blot: 1:50-400Immunohistochemistry (frozen): 1:50-500Immunohistochemistry (paraffin): 1:10-100Immunocytochemistry: 1:50-500Optimal 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
仕様
Size：200ul
Host：rabbit
Source Antibody：mouse
Grade：Affinity Purified
Purity：Purified by immunoaffinity chromatography.
Form：Supplied as a liquid in PBS, pH 7.4, 0.02% sodium azide, 50% glycerol.
Specificity：Recognizes mouse GaA.
Isotype：IgG
Calc Applications Abbrev：E IC IHC WB
Calc Crossreactivity：Mo
Immunogen：Recombinant protein corresponding to Glu70-Phe245 of mouse GaA, fused to two N-terminal Tags, His-Tag and T7-tag, expressed in E. coli.
