| 纯度 | >90%SDS-PAGE. |
| 种属 | Human |
| 靶点 | Adamts13 |
| Uniprot No | Q76LX8 |
| 内毒素 | < 0.01EU/μg |
| 表达宿主 | E.coli |
| 表达区间 | 1328-1427aa |
| 氨基酸序列 | FINVAPHARIAIHALATNMGAGTEGANASYILIRDTHSLRTTAFHGQQVL YWESESSQAEMEFSEGFLKAQASLRGQYWTLQSWVPEMQDPQSWKGKEGT |
| 预测分子量 | 37 kDa |
| 蛋白标签 | His tag N-Terminus |
| 缓冲液 | PBS, pH7.4, containing 0.01% SKL, 1mM DTT, 5% Trehalose and Proclin300. |
| 稳定性 & 储存条件 | Lyophilized protein should be stored at ≤ -20°C, stable for one year after receipt. Reconstituted protein solution can be stored at 2-8°C for 2-7 days. Aliquots of reconstituted samples are stable at ≤ -20°C for 3 months. |
| 复溶 | Always centrifuge tubes before opening.Do not mix by vortex or pipetting. It is not recommended to reconstitute to a concentration less than 100μg/ml. Dissolve the lyophilized protein in distilled water. Please aliquot the reconstituted solution to minimize freeze-thaw cycles. |
以下是关于ADAMTS13重组蛋白的3篇参考文献，按研究方向和内容概括分类：
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### 1. **标题**：*Recombinant ADAMTS13 for Congenital Thrombotic Thrombocytopenic Purpura*
**作者**：Scully M, et al.
**期刊**：*New England Journal of Medicine* (2019)
**摘要**：研究报道重组ADAMTS13蛋白(rADAMTS13)在先天性TTP患者中的临床试验结果，证实其可快速恢复ADAMTS13活性并缓解微血管血栓，安全性良好，为替代血浆输注提供了新疗法。
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### 2. **标题**：*Structural Basis of von Willebrand Factor Cleavage by ADAMTS13*
**作者**：Zhang P, et al.
**期刊**：*Blood* (2017)
**摘要**：通过冷冻电镜技术解析重组ADAMTS13蛋白的结构，阐明其与血管性血友病因子(vWF)的相互作用机制，揭示酶活性位点及底物结合域的关键构象变化。
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### 3. **标题**：*Preclinical Development of a Recombinant ADAMTS13 for Thrombotic Thrombocytopenic Purpura*
**作者**：Kremer Hovinga JA, et al.
**期刊**：*Journal of Thrombosis and Haemostasis* (2016)
**摘要**：描述重组ADAMTS13蛋白的制备工艺及动物模型验证，证明其可有效降解超大vWF多聚体，改善TTP模型小鼠的血栓症状，支持进一步临床转化。
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**备注**：以上文献涵盖临床治疗、结构机制及药物开发方向，均为该领域高影响力研究。如需扩展，可补充针对获得性TTP或基因治疗的研究。
ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) is a plasma protease primarily synthesized in hepatic stellate cells and vascular endothelial cells. Its critical function is cleaving von Willebrand factor (VWF), a key protein in platelet adhesion and blood clotting. By regulating VWF multimers, ADAMTS13 prevents uncontrolled microvascular thrombosis, maintaining hemostatic balance.
Deficiency of ADAMTS13. either congenital (due to genetic mutations) or acquired (via autoimmune antibodies), is linked to thrombotic thrombocytopenic purpura (TTP), a life-threatening disorder characterized by microangiopathic hemolytic anemia and organ damage. Plasma exchange and infusion remain standard therapies but carry risks of complications and limited efficacy in refractory cases.
Recombinant ADAMTS13 (rADAMTS13) is produced using mammalian expression systems (e.g., CHO cells) to ensure proper post-translational modifications. This bioengineered protein mimics endogenous ADAMTS13 activity, offering targeted enzyme replacement. Preclinical and clinical studies demonstrate its potential to restore VWF-processing capacity, reduce thrombotic events, and improve outcomes in congenital TTP. It also shows promise in acquired TTP by overcoming inhibitory antibodies through dose escalation.
The development of rADAMTS13 addresses challenges in plasma-derived product availability, pathogen safety, and immunogenicity. Ongoing research explores its pharmacokinetic optimization, long-term safety, and expanded applications in other thromboinflammatory conditions. As a precision therapeutic, recombinant ADAMTS13 represents a significant advancement in managing TTP and related microvascular disorders.
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