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 Western Blot, Immunohistochemistry and ELISA.
Other applications have not been tested.
Recommended Dilutions:Immunohistochemistry: Frozen and formalin fixed and paraffin embedded tissuesOptimal dilutions to be determined by the researcher.
Storage and Stability:Store product at 4°C if to be used immediately within two weeks.
For long-term storage, aliquot to avoid repeated freezing and thawing and store at -20°C.
Aliquots are stable at -20°C for 12 months after receipt.
Dilute required amount only prior to immediate use.
Further dilutions can be made in assay buffer.
For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
Note: Applications are based on unconjugated antibody.
仕様
Size：200ul
Host：rabbit
Source Antibody：human
Grade：Affinity Purified
Purity：Purified by Protein A and peptide affinity chromatography.
Form：Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Biotin.
Specificity：Recognizes human GAA.
Isotype：IgG
Calc Applications Abbrev：E IHC WB
Calc Crossreactivity：Hu
Immunogen：KLH-conjugated synthetic peptide mapping to a fragment of residues within amino acids 174-203 in the N-terminal region of human GAA.
