

| Catalog No. | HY377014 | ||||||||
|---|---|---|---|---|---|---|---|---|---|
| Species reactivity | Human | ||||||||
| Applications | ELISA, IHC, WB | ||||||||
| Host species | Rabbit | ||||||||
| Isotype | IgG | ||||||||
| Clonality | Polyclonal | ||||||||
| Immunogen | E. coli - derived recombinant Human GAA (Pro595-Gly770). | ||||||||
| Target | GAA, Lysosomal alpha-glucosidase, Acid maltase, Aglucosidase alfa | ||||||||
| Purification | Purified by antigen affinity column. | ||||||||
| Accession | P10253 | ||||||||
| Form | Liquid | ||||||||
| Storage buffer | 0.01M PBS, pH 7.4, 50% Glycerol, 0.05% Proclin 300. Please refer to the specific buffer information in the hardcopy of datasheet or the lot-specific COA. |
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| Product Usage Information |
| ||||||||
| Stability and Storage | Use a manual defrost freezer and avoid repeated freeze thaw cycles. Store at 2 to 8°C for frequent use. Store at -20 to -80°C for twelve months from the date of receipt. | ||||||||
| Background | Lysosomal alpha-glucosidase (GAA/Aglucosidase alfa) is a ~105 kDa protein. Essential for the degradation of glycogen in lysosomes. Has highest activity on alpha-1,4-linked glycosidic linkages, but can also hydrolyze alpha-1,6-linked glucans. 1. Hermans, MM. et al. (2004) Human mutation 23, 47-56. PMID: 14695532 2. Pittis, MG. et al. (2008) Human mutation 29, E27-36. PMID: 18429042 3. Hermans, MM. et al. (1991) The Journal of biological chemistry 266, 13507-12. PMID: 1856189 4. Boerkoel, CF. et al. (1995) American journal of human genetics 56, 887-97. PMID: 7717400 5. Roig-Zamboni, V. et al. (2017) Nature communications 8, 1111. PMID: 29061980 | ||||||||
| Note | For research use only. |

Western blot analysis was performed using anti-GAA polyclonal antibody at 1ug/mL on recombinant GAA (Catalog No. HY377012).




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