Desmosomes and disease: Pemphigus and bullous impetigo

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

Research output: Contribution to journalReview article

103 Citations (Scopus)

Abstract

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.

Original languageEnglish
Pages (from-to)536-543
Number of pages8
JournalCurrent Opinion in Cell Biology
Volume16
Issue number5
DOIs
Publication statusPublished - 2004 Oct 1

Keywords

  • Dsg
  • ET
  • FS
  • IgG
  • MP
  • PF
  • PV
  • SSSS
  • desmoglein
  • exfoliative toxin
  • fogo selvagem
  • immunoglobulin G
  • mAb
  • methylprednisolone
  • monoclonal antibody
  • pemphigus foliaceus
  • pemphigus vulgaris
  • staphylococcal scalded skin syndrome

ASJC Scopus subject areas

  • Cell Biology

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