| 货号 | MAB2764-SP |
| 别名 | F8VWF; VWD; VWF von Willebrand factor; VWF | 全称 | von Willebrand Factor A2 Domain |
| 反应种属 | Human |
| 应用 | Western Blot(1 µg/mL) |
| 目标/特异性 | Detects human vWF-A2 in direct ELISAs and Western blots. This antibody detects an epitope C-terminal to the ADAMTS13 cleavage site between aa 1606-1665. |
| 使用方法 | Western Blot: 1 µg/mL |
| 来源 | Reconstitute at 0.5 mg/mL in sterile PBS. |
| 产品组分 |
| 供应商 | R&D Systems |
| Entrez Gene IDs | 7450 (Human); 22371 (Mouse); 116669 (Rat) |
| 纯化方式 | Protein A or G purified from hybridoma culture supernatant |
| 免疫原 | E. coli-derived recombinant human vWF-A2 Asp1498-Val1665 Accession # P04275 |
| 生物活性 | Human |
| 标记 | Unconjugated |
| 溶解方法 | Reconstitute at 0.5 mg/mL in sterile PBS. |
| 背景 | von Willebrand Factor (vWF) is a large, multimeric glycoprotein made by endothelial cells and megakaryocytes. The pre-pro-vWF protein contains 2813 amino acids (aa), which consists of 22 aa signal peptide, 741 aa propeptide, and mature vWF monomer of 2050 aa (1-4). The pro-vWF undergoes dimerization in the endoplasmic reticulum (ER) through C-terminal “cysteine-knot” (CK) domain. The pro-vWF dimmers are transported to Golgi and form multimers by forming disulfide bond in amino-terminal region of the mature form. The proteolytic processing of pro-region also occurs in Golgi. The matured vWF is stored in Weibel-Pallade bodies in endothelial cells and granules in megakaryocytes and platelets. The unusually-large vWF (ulvWF) multimers released from cells are very efficient in binding to platelets to form thrombus. The population of these highly active ulvWF multimers is controlled by a specific protease, ADAMTS13, which cleaves between residues Tyr1605 and Met1606 in the A2 domain of vWF. In the plasma, vWF appears as a series of large and intermediate multimers with molecular masses from several thousand to 500 kDa. vWF also performs hemostatic functions (3-5). In a high shear-stressed environment, vWF undergoes conformational change to expose a binding site for glycoprotein Ib alpha. As a result, vWF facilitates aggregation of platelets. In addition to platelet binding, vWF binds coagulation factor VIII to increase the lifetime of FVIII in plasma. The purified rhvWF-A2 contains the A2 domain of vWF. |
| 运输条件 | Blue Ice |
| 存放说明 | 4℃ |
| 参考文献 |
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