Desmosomes and disease: Pemphigus and bullous impetigo

Aimee S. Payne, Yasushi Hanakawa, Masayuki Amagai, John R. Stanley

研究成果: Review article

103 引用 (Scopus)

抜粋

Desmosomal cadherins are the pathophysiologic targets of autoimmune or toxin-mediated disruption in the human diseases pemphigus and bullous impetigo (including its generalized form, called staphylococcal scalded skin syndrome). Experiments exploiting the production of both pathogenic and nonpathogenic antidesmoglein antibodies in pemphigus patients' sera have afforded data that make an invaluable contribution towards identifying the functional domains of the desmogleins involved in intercellular adhesion. Conformational epitopes of antidesmoglein autoantibodies in pemphigus patients' sera and the specific cleavage site of desmoglein 1 by exfoliative toxin have been identified, implicating the N-terminal extracellular domains of the desmogleins as critical regions for controlling intercellular adhesion. Furthermore, the development of active autoimmune mouse models for pemphigus allows in vivo characterization of the disease and its pathogenesis. These studies offer new insight into the potential mechanisms of acantholysis in pemphigus and staphylococcal-associated blistering disease, with implications for the role of desmogleins in desmosomal structure and function.

元の言語English
ページ(範囲)536-543
ページ数8
ジャーナルCurrent Opinion in Cell Biology
16
発行部数5
DOI
出版物ステータスPublished - 2004 10 1

ASJC Scopus subject areas

  • Cell Biology

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