Document Detail

Antibodies against desmoglein 3 (pemphigus vulgaris antigen) are present in sera from patients with paraneoplastic pemphigus and cause acantholysis in vivo in neonatal mice.
MedLine Citation:
PMID:  9710446     Owner:  NLM     Status:  MEDLINE    
Paraneoplastic pemphigus (PNP) is an autoimmune blistering disease that occurs in association with underlying neoplasms. Patients with PNP develop characteristic IgG autoantibodies directed against multiple antigens, most of which have been identified as cytoplasmic proteins of the plakin family (desmoplakin I, II, BPAG1, envoplakin, and periplakin). This study identified cell surface target antigens of PNP. We focused on desmoglein (Dsg) 3 and Dsg1, the autoantigens of pemphigus vulgaris and pemphigus foliaceus. ELISA using baculovirus-expressed recombinant Dsgs (rDsg3, rDsg1) has revealed that 25 out of 25 PNP sera tested were positive against Dsg3 and 16 of 25 were positive against Dsg1. All of 12 PNP sera tested immunoprecipitated Dsg3. Removal of anti-Dsg3 autoantibodies by immunoadsorption was sufficient to eliminate the ability of PNP sera to induce cutaneous blisters in neonatal mice in vivo. Furthermore, anti-Dsg3-specific antibodies that were affinity purified from PNP sera were proven to be pathogenic and caused blisters in neonatal mice. These findings indicate that Dsg3 and Dsg1 are the cell surface target antigens in PNP and that IgG autoantibodies against Dsg3 in PNP sera play a pathogenic role in inducing loss of cell adhesion of keratinocytes and causing blister formation.
M Amagai; T Nishikawa; H C Nousari; G J Anhalt; T Hashimoto
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  The Journal of clinical investigation     Volume:  102     ISSN:  0021-9738     ISO Abbreviation:  J. Clin. Invest.     Publication Date:  1998 Aug 
Date Detail:
Created Date:  1998-09-16     Completed Date:  1998-09-16     Revised Date:  2009-11-18    
Medline Journal Info:
Nlm Unique ID:  7802877     Medline TA:  J Clin Invest     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  775-82     Citation Subset:  AIM; IM    
Department of Dermatology, Keio University School of Medicine, Tokyo 160-8582, Japan.
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MeSH Terms
Acantholysis / chemically induced,  immunology*
Animals, Newborn
Autoantibodies / blood*,  toxicity
Autoantigens / immunology*
Blister / chemically induced
Cadherins / immunology*
Desmoglein 1
Desmoglein 3
Paraneoplastic Syndromes / etiology,  immunology*
Pemphigus / etiology,  immunology*
Skin / pathology
Reg. No./Substance:
0/Autoantibodies; 0/Autoantigens; 0/Cadherins; 0/DSG3 protein, human; 0/Desmoglein 1; 0/Desmoglein 3

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