Document Detail

Desmoglein 3, its pathogenecity and a possibility for therapeutic target in pemphigus vulgaris.
MedLine Citation:
PMID:  23294403     Owner:  NLM     Status:  MEDLINE    
INTRODUCTION: Desmoglein 3 (Dsg3) is one of desmosomal cadherins and functions in epidermal keratinocyte adhesion. IgG anti-Dsg3 autoantibodies are detected in pemphigus vulgaris, an autoimmune bullous disease showing blisters and erosions on the skin and oral mucosa. Other types of pemphigus also show anti-Dsg3 antibodies. Genetic disease of Dsg3 has not been reported.
AREAS COVERED: Many in vitro and in vivo studies have indicated pathogenic role of anti-Dsg3 antibodies. Blisters in pemphigus vulgaris are thought to be developed by loss of keratinocyte adhesions by binding of anti-Dsg3 antibodies to Dsg3 through steric hindrance, internalization of Dsg3, changes in molecular integrity or signal transduction. There are pathogenic and nonpathogenic anti-Dsg3 antibodies reactive with different epitopes. Recent studies of pemphigus vulgaris include existence of non-Dsg3 autoantibodies, B cells and T cells reactive with Dsg3, involvement of TNF-α and IL-1 and activation of intracellular signaling.
EXPERT OPINION: Although systemic corticosteroids and immunosuppressive agents are mainstays for treatment of pemphigus, intravenous immunoglobulin, plasmapheresis, immunoadsorption, rituximab and TNF-α inhibitors are emerging. Anti-Dsg3 antibody-targeting therapies are reported in mouse model, but they are not yet available clinically. Clarification of pathogenic role of anti-Dsg3 antibodies in pemphigus should provide us with safer and more effective therapies.
Hiroshi Koga; Daisuke Tsuruta; Bungo Ohyama; Norito Ishii; Takahiro Hamada; Chika Ohata; Minao Furumura; Takashi Hashimoto
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Review     Date:  2013-01-08
Journal Detail:
Title:  Expert opinion on therapeutic targets     Volume:  17     ISSN:  1744-7631     ISO Abbreviation:  Expert Opin. Ther. Targets     Publication Date:  2013 Mar 
Date Detail:
Created Date:  2013-02-13     Completed Date:  2013-07-30     Revised Date:  2013-11-06    
Medline Journal Info:
Nlm Unique ID:  101127833     Medline TA:  Expert Opin Ther Targets     Country:  England    
Other Details:
Languages:  eng     Pagination:  293-306     Citation Subset:  IM    
Kurume University School of Medicine, and Kurume University Institute of Cutaneous Cell Biology, Department of Dermatology, 67 Asahimachi, Kurume, Fukuoka, 830-0011, Japan.
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MeSH Terms
Antibodies / immunology
Cell Adhesion
Desmoglein 3 / antagonists & inhibitors,  immunology,  metabolism*
Pemphigus / drug therapy,  metabolism*
Reg. No./Substance:
0/Antibodies; 0/Desmoglein 3

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