ADAMTS13抗体;ADAMTS13 Antibody—艾普蒂

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     The image is immunohistochemistry of paraffin-embedded Human cervical cancer tissue using P11426(ADAMTS13 Antibody) at dilution 1/25. (Original magnification: ×200)    

  
  

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     The image is immunohistochemistry of paraffin-embedded Human gastric cancer tissue using P11426(ADAMTS13 Antibody) at dilution 1/25. (Original magnification: ×200)    

  
  

ADAMTS13 is a plasma metalloprotease primarily responsible for cleaving von Willebrand factor (VWF) multimers, regulating their size and preventing spontaneous platelet aggregation. Autoantibodies targeting ADAMTS13 are central to the pathogenesis of immune-mediated thrombotic thrombocytopenic purpura (iTTP), a rare, life-threatening thrombotic microangiopathy. These antibodies, typically IgG, neutralize ADAMTS13 activity or accelerate its clearance, leading to ultra-large VWF multimers that promote microvascular thrombosis.

Most anti-ADAMTS13 antibodies are inhibitory, directly blocking enzyme-substrate interaction or binding to critical domains like the spacer region. Non-inhibitory antibodies may enhance proteolytic degradation or disrupt secretion. Their development is linked to autoimmune dysregulation, though triggers remain unclear. Genetic factors (e.g., HLA-DRB1*11) and environmental stimuli (infections, pregnancy) may contribute.

Laboratory diagnosis relies on detecting severe ADAMTS13 deficiency (<10% activity) with anti-ADAMTS13 antibodies. Treatment involves plasma exchange, immunosuppression (rituximab, corticosteroids), and caplacizumab. Persistent antibodies correlate with relapse risk, necessitating monitoring. Research continues to map epitopes and develop targeted therapies. Understanding these antibodies enhances management of iTTP and related disorders.