| WB | 1/1000-1/2000 | Human,Mouse,Rat |
| IF | 咨询技术 | Human,Mouse,Rat |
| IHC | 咨询技术 | Human,Mouse,Rat |
| ICC | 技术咨询 | Human,Mouse,Rat |
| FCM | 1/20-1/100 | Human,Mouse,Rat |
| Elisa | 咨询技术 | Human,Mouse,Rat |
| Aliases | ADAMTS13; ADAM-TS; ADAM-TS13; ADAMTS-13; ADAM-TS 13; C9orf8; TTP; VWF-CP; VWFCP;;ADAMTS13 |
| WB Predicted band size | 154 kDa |
| Host/Isotype | Rabbit IgG |
| Antibody Type | Primary antibody |
| Storage | Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles. |
| Species Reactivity | Human |
| Immunogen | A synthesized peptide derived from human ADAMTS13 |
| Formulation | Purified antibody in PBS with 0.05% sodium azide,0.05% BSA and 50% glycerol. |
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以下是关于ADAMTS13抗体的3-4篇参考文献，按文献名称、作者和摘要内容概括列举：
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1. **文献名称**：*Antibodies to ADAMTS13 in acquired thrombotic thrombocytopenic purpura*
**作者**：Zheng XL, Wu HM, Crawford J, et al.
**摘要**：该研究分析了获得性血栓性血小板减少性紫癜(TTP)患者中ADAMTS13抗体的特性，发现这些抗体通过抑制ADAMTS13的蛋白酶活性或加速其清除，导致血管性血友病因子(vWF)异常聚集，进而引发微血管血栓形成。
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2. **文献名称**：*ADAMTS13 and anti-ADAMTS13 antibodies in patients with thrombotic thrombocytopenic purpura*
**作者**：Fujimura Y, Matsumoto M.
**摘要**：研究探讨了日本TTP患者中ADAMTS13自身抗体的临床意义，发现抗体水平与疾病严重程度相关，并提出了基于ADAMTS13活性检测和抗体滴度的诊断与治疗策略。
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3. **文献名称**：*Rituximab for refractory thrombotic thrombocytopenic purpura: a case report and review of the role of ADAMTS13 antibody testing*
**作者**：Scully M, Cohen H, Cavenagh J, et al.
**摘要**：通过病例分析和文献综述，总结了利妥昔单抗治疗抗体介导的难治性TTP的效果，强调ADAMTS13抗体检测在指导靶向治疗(如B细胞耗竭疗法)中的重要性。
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4. **文献名称**：*ADAMTS13 autoantibodies in immune-mediated thrombotic thrombocytopenic purpura*
**作者**：Kremer Hovinga JA, Lämmle B.
**摘要**：综述了ADAMTS13自身抗体的病理机制，包括IgG亚型分布、抗原表位识别及其与疾病复发风险的关系，同时讨论了新型免疫抑制剂在清除抗体中的应用前景。
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以上文献涵盖抗体机制、临床关联及治疗方向，均发表于高影响力血液学期刊(如《Blood》《Journal of Thrombosis and Haemostasis》)。
ADAMTS13 is a plasma metalloprotease primarily synthesized in the liver and endothelial cells. Its critical role lies in cleaving ultra-large von Willebrand factor (VWF) multimers into smaller, less adhesive forms, preventing spontaneous platelet aggregation and microvascular thrombosis. Antibodies against ADAMTS13. typically IgG autoantibodies, are central to the pathogenesis of immune-mediated thrombotic thrombocytopenic purpura (iTTP), a rare but life-threatening thrombotic microangiopathy. These antibodies either inhibit ADAMTS13 enzymatic activity or accelerate its clearance, leading to a severe deficiency (<10% activity) and accumulation of uncleaved VWF multimers. This promotes platelet clumping, microthrombi formation, and end-organ ischemia.
Most anti-ADAMTS13 antibodies target the spacer domain (C-terminal regions) or cysteine-rich domains of the enzyme, disrupting its interaction with VWF. Their production is often idiopathic, though occasionally linked to autoimmune disorders, infections, or malignancies. Diagnosis combines clinical presentation (thrombocytopenia, hemolytic anemia, neurological/renal dysfunction) with laboratory confirmation of ADAMTS13 deficiency and antibody detection via ELISA or functional assays.
Treatment focuses on removing antibodies through plasma exchange and suppressing their production using immunosuppressants (e.g., corticosteroids, rituximab). Early intervention is crucial to reduce mortality. Research continues to explore antibody epitope specificity, long-term remission predictors, and targeted therapies to mitigate autoimmune responses against ADAMTS13.
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