Desmoglein3抗体;Desmoglein3 Antibody—艾普蒂 新品

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     Western blot analysis of Desmoglein 3 expression in A431 cell lysate.    

  
  

Desmoglein 3 (DSG3) is a transmembrane glycoprotein belonging to the desmoglein family, primarily expressed in stratified squamous epithelia, particularly in the basal and immediate suprabasal layers of the skin and mucous membranes. It plays a critical role in cell-cell adhesion through desmosomes, which maintain tissue integrity. DSG3 antibodies are autoantibodies targeting this protein, most notably associated with pemphigus vulgaris (PV), a rare but severe autoimmune blistering disorder. In PV, IgG autoantibodies bind to DSG3. disrupting desmosomal adhesion and causing acantholysis—separation of epidermal cells—leading to painful mucosal and cutaneous blisters.

DSG3 antibodies are predominantly of the IgG4 subclass, though IgG1 and other subclasses may contribute. Their pathogenicity is confirmed by passive transfer experiments, where injecting these antibodies into mice reproduces blistering. Diagnosis relies on detecting DSG3 antibodies via ELISA or indirect immunofluorescence, with DSG1 antibodies often co-present in mucocutaneous PV. Research suggests epitope-specific responses, where antibodies targeting certain DSG3 domains correlate with disease severity.

Treatment focuses on immunosuppression (e.g., corticosteroids, rituximab) to reduce antibody production. Recent studies explore therapies targeting B cells, plasma cells, or DSG3-specific antibodies directly. DSG3 antibody research also extends to paraneoplastic pemphigus and drug-induced pemphigus, broadening understanding of autoimmune desmosome disruption.