Human autoantibodies against HD1/plectin in paraneoplastic pemphigus

摘要: Paraneoplastic pemphigus (PNP) is an autoimmune blistering disease that occurs in association with underlying neoplasms. PNP patients develop characteristic autoantibodies directed against multiple antigens, mostly identified as members of the plakin family cytoplasmic proteins (desmoplakin I and II, bullous pemphigoid antigen I, envoplakin, periplakin). HD1/plectin, another member family, has not previously been detected complex, which may relate to practical difficulties associated its large size (molecular weight ≈ 500 kDa). In this study, a combination immunoprecipitation immunoblot used demonstrate HD1/plectin also recognized by sera from patients. Thirteen 16 tested were positive for compared none 43 control (11 vulgaris, 11 foliaceus, pemphigoid, 10 normal individuals). Combined our recent finding desmoglein 3 1 are cell surface target antigens PNP, demonstration plectin/HD1 component complex confirms molecules.