

| 货号 | HY377036 |
|---|---|
| 品牌 | abinScience |
| 种属反应性 | Human |
| 应用 | ELISA, Bioactivity: FACS, Functional assay, Research in vivo |
| 宿主 | Human |
| 同种型 | IgG1-kappa (fused with Homo sapiens GAA/glucosidase alpha) |
| 生物活性 | Lysosomal α-Glucosidase is a γ-amylase that is essential for the degradation of glycogen in lysosomes [1] [2]. |
| 表达系统 | Mammalian cells |
| 克隆类型 | Monoclonal |
| 靶标 | Equilibrative nitrobenzylmercaptopurine riboside-insensitive nucleoside transporter, 36 kDa nucleolar protein HNP36, ENT2, Equilibrative nucleoside transporter 2, SLC29A2, Hydrophobic nucleolar protein, 36 kDa, Equilibrative NBMPR-insensitive nucleoside transporter, Nucleoside transporter, ei-type, Delayed-early response protein 12, Solute carrier family 29 member 2, HNP36, DER12, GAA, Lysosomal alpha-glucosidase, Acid maltase, Aglucosidase alfa |
| 内毒素水平 | < 10 EU/mg |
| 纯度 | >95% purity as determined by SDS-PAGE. |
| 纯化方式 | Protein A/G purified from cell culture supernatant. |
| Accession号 | Q14542 & P10253 |
| 状态 | Liquid |
| 保存溶液 | 0.01M PBS pH 7.4 Please refer to the specific buffer information in the hardcopy of datasheet or the lot-specific COA. |
| 稳定性和存储 | 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. |
| 别名 | VAL-1221 |
| 背景 | Clervonafusp alfa (VAL-1221) is a fusion protein targeting both cytosolic and lysosomal glycogen. Clervonafusp alfa is comprised of the Fab portion of a cell-penetrating antibody and recombinant human acid alpha glucosidase (rhGAA), the former utilizing the nucleoside transporter ENT-2 to gain access to the cytosol, and the latter enters lysosomes via mannose-6-phosphate receptors (M6PRs). Clervonafusp alfa can be used for late-onset Pompe disease research. |
| 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. [HY377036] 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. [HY377036] |
| Note | For research use only. Not suitable for clinical or therapeutic use. |

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