| Catalog Code: | H00002548-B01 |
| Product Type: | Primary Antibody |
| Product Name: | GAA Antibody |
| List Price: | 295 |
| Clonality: | Polyclonal |
| Gene ID: | 2548 |
| Host: | Mouse |
| Product Description: | Mouse Polyclonal anti-GAA - glucosidase, alpha; acid (Pompe disease, glycogen storage disease type II), MaxPab Antibody |
| Cross Reactivity: | Human. |
| Species Summary: | Hu(+) |
| Background: | 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. |
| Alternate Names: | anti-LYAG antibody |
| Immunogen: | GAA (-, 1 a.a. ~ 952 a.a) full-length human protein. |
| Epitope: | MGVRHPPCSHRLLAVCALVSLATAALLGHILLHDFLLVPRELSGSSPVLEETHPAHQQGASRPGPRDAQAHPGRPRAVPTQCDVPPNSRFDCAPDKAITQEQCEARGCCYIPAKQGLQGAQMGQPWCFFPPSYPSYKLENLSSSEMGYTATLTRTTPTFFPKDILTLRLDVMMETENRLHFTIKDPANRRYEVPLETPRVHSRAPSPLYSVEFSEEPFGVIVHRQLDGRVLLNTTVAPLFFADQFLQLSTSLPSQ ... |
| Preservative: | No Preservative |
| Packaging: | 0.05 ml of mouse antisera with 50% glycerol. |
| PackageSize: | 0.05 ml |
| Uses: | Antibody Reactive Against Recombinant Protein with GST tag on ELISA and Western Blot and also on transfected lysate in western blot. GST tag alone is used as a negative control. |
| Application Summary: | Western Blot 1:500, ELISA |
| Storage: | Aliquot and store at -20C or -80C. Avoid freeze-thaw cycles. |
| WebLink: | www.novusbio.com/data_sheet/index ... |