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| カタログ番号 | HY377306 |
|---|---|
| 種反応性 | Human |
| アプリケーション | ELISA, Functional assay, Research in vivo |
| クローン性 | Monoclonal |
| 生物学的活性 | Lysosomal α-Glucosidase is a γ-amylase that is essential for the degradation of glycogen in lysosomes [1] [2]. |
| 発現システム | Mammalian cells |
| ターゲット | GAA, Lysosomal alpha-glucosidase, Acid maltase, Aglucosidase alfa |
| エンドトキシンレベル | < 10 EU/mg |
| 純度 | >95% purity as determined by SDS-PAGE. |
| 精製 | Protein A/G purified from cell culture supernatant. |
| アクセッション番号 | P10253 |
| 形態 | Liquid |
| 保存バッファー | 0.01M PBS pH 7.4 データシートのハードコピーまたはロット固有のCOAに記載された具体的なバッファー情報を参照してください。 |
| 安定性と保存 | 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. |
| 別名 | GZ-402666, avalglucosidasealfa-ngpt |
| 背景 | 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 |
| 参考文献 | 1. Hermans MM, et al. Human lysosomal alpha-glucosidase: functional characterization of the glycosylation sites. The Biochemical journal. 1993 Feb 01;289 (Pt 3)(Pt 3):681-6. [HY377306] 2. Hoefsloot LH, et al. Primary structure and processing of lysosomal alpha-glucosidase; homology with the intestinal sucrase-isomaltase complex. The EMBO journal. 1988 Jun;7(6):1697-704. [HY377306] |
| 注意事項 | For research use only. Not suitable for clinical or therapeutic use. |
SDS-PAGE for Research Grade Nexviazyme/Avalglucosidase alfa.
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