Human Autoantibodies against HD1/Plectin in Paraneoplastic Pemphigus

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 of cytoplasmic proteins (desmoplakin I and...

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Veröffentlicht in:Journal of investigative dermatology 1999-02, Vol.112 (2), p.153-156
Hauptverfasser: Proby, Charlotte, Fujii, Yoshiko, Nishikawa, Takeji, Amagai, Masayuki, Owaribe, Katsushi
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container_start_page 153
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creator Proby, Charlotte
Fujii, Yoshiko
Nishikawa, Takeji
Amagai, Masayuki
Owaribe, Katsushi
description 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 of cytoplasmic proteins (desmoplakin I and II, bullous pemphigoid antigen I, envoplakin, and periplakin). HD1/plectin, another member of the plakin family, has not previously been detected in the characteristic PNP antigen complex, which may relate to practical difficulties associated with its large size (molecular weight ≈ 500 kDa). In this study, a combination of immunoprecipitation and immunoblot is used to demonstrate that HD1/plectin is also recognized by sera from PNP patients. Thirteen of 16 PNP sera tested were positive for HD1/plectin compared with none of 43 control sera (11 pemphigus vulgaris, 11 pemphigus foliaceus, 11 bullous pemphigoid, and 10 normal individuals). Combined with our recent finding that desmoglein 3 and desmoglein 1 are cell surface target antigens in PNP, this demonstration of plectin/HD1 as another component of the antigen complex in PNP confirms that PNP is an autoimmune disease against desmoglein and plakin family molecules.
doi_str_mv 10.1046/j.1523-1747.1999.00498.x
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source MEDLINE; Elektronische Zeitschriftenbibliothek - Frei zugängliche E-Journals; Alma/SFX Local Collection
subjects Autoantibodies - immunology
autoimmunity
Biological and medical sciences
Bullous diseases of the skin
Dermatology
desmoglein
Humans
Immunoblotting
Intermediate Filament Proteins - immunology
Medical sciences
Paraneoplastic Syndromes - blood
Paraneoplastic Syndromes - immunology
Pemphigus - blood
Pemphigus - immunology
plakins
Plectin
Precipitin Tests
title Human Autoantibodies against HD1/Plectin in Paraneoplastic Pemphigus
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