CollagenXVII抗体;CollagenXVII Antibody—艾普蒂 新品

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     All lanes use the Antibody at 1:2K dilution for 1 hour at room temperature.    

  
  

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     All lanes use the Antibody at 1:2K dilution for 1 hour at room temperature.    

  
  

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     Western blot analysis of Collagen XVII expression in Human skin lysate.    

  
  

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     Immunofluorescent analysis of A431 cells, using Collagen XVII Antibody .    

  
  

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     Immunohistochemical analysis of paraffin-embedded mouse skin, using Collagen XVII Antibody.    

  
  

Collagen XVII, also known as BP180 or BPAG2. is a transmembrane protein primarily expressed in hemidesmosomes of basal keratinocytes, where it plays a critical role in maintaining dermal-epidermal adhesion. It consists of a collagenous extracellular domain and a cytoplasmic N-terminal domain, functioning as a structural component and signaling mediator. Autoantibodies targeting Collagen XVII are central to the pathogenesis of autoimmune blistering diseases, particularly bullous pemphigoid (BP) and mucous membrane pemphigoid. In BP, IgG autoantibodies bind to the NC16A domain of Collagen XVII, triggering inflammatory cascades that lead to blister formation.

Diagnostically, Collagen XVII antibodies are detected via immunofluorescence, ELISA, or immunoblotting, aiding in disease confirmation and monitoring. Research also explores their role in rare genetic disorders like junctional epidermolysis bullosa, where Collagen XVII mutations cause skin fragility. Therapeutic strategies targeting these antibodies, such as immunoadsorption or biologics, are under investigation. Recent studies focus on epitope mapping, antibody subclasses, and their correlation with disease severity, enhancing understanding of pathophysiology and treatment optimization. Additionally, Collagen XVII-deficient animal models have been instrumental in studying skin homeostasis and repair mechanisms.