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GIORNALE ITALIANO DI DERMATOLOGIA E VENEREOLOGIA

A Journal on Dermatology and Sexually Transmitted Diseases


Official Journal of the Italian Society of Dermatology and Sexually Transmitted Diseases
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Giornale Italiano di Dermatologia e Venereologia 2007 August;142(4):337-43

language: English

Desmoglein 3 in the pathogenesis of pemphigus

Faurschou A., Gniadecki R.

Department of Dermatology Bispebjerg Hospital, Copenhagen, Denmark


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The involvement of antidesmoglein antibodies in the development of pemphigus vulgaris and pemphigus foliaceus has been extensively documented, but how these antibodies participate in the pathogenesis of pemphigus is still debated. The theory that antidesmoglein antibodies are the sole determinant of the pathogenic activity present in the pemphigus sera directly disrupting desmosomes and producing acantholysis is no longer valid. Antidesmoglein antibodies are more likely to interact with the membrane pool of nondesmosomal desmogleins causing their clustering and endocytosis. This depletion of desmoglein from the surface of the membrane may have two important consequences: 1) interference with the synthesis of new desmosomes and, more importantly, 2) disruption of the association with armadillo and plakin partner proteins. The latter event could directly induce several signalling events, in particular reorganisation of intermediate and actin filaments and cell retraction, activation of p38 and heat shock proteins, and activation of several pro-apoptotic pathways culminating in formation of an acantholytic cell.

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