title: Pemphigus, a pathomechanism of acantholysis.
abstract: Autoantibodies to the desmosomal proteins desmoglein 1 and 3 cause pemphigus foliaceus and pemphigus vulgaris, which are characterised by keratinocyte dissociation (acantholysis) and intraepidermal blister formation. The passive transfer of pathogenic anti-desmoglein antibodies induces blisters in mice in vivo and the loss of keratinocyte adhesion in vitro. The pathogenetic mechanisms of acantholysis due to anti-desmoglein autoantibodies are not fully understood. However, recent studies have revealed that signalling-dependent and signalling-independent pathways are operative in the loss of cell adhesion. In this review, we focus on the pathomechanism of acantholysis due to autoantibodies to desmogleins and recent therapeutic approaches.
authors.affiliation: Department of Dermatology, Kyushu University, Fukuoka, Japan.
authors.forename: Masutaka
authors.initials: M
authors.lastname: Furue
authors.affiliation: Department of Dermatology, St Marianna University School of Medicine, Kawasaki, Japan.
authors.forename: Takafumi
authors.initials: T
authors.lastname: Kadono
mesh_terms: D000051:Acantholysis / Q000276:immunology*; D000818:Animals; D001323:Autoantibodies / Q000276:immunology*; D051183:Desmoglein 1 / Q000276:immunology / Q000378:metabolism*; D051184:Desmoglein 3 / Q000276:immunology / Q000378:metabolism*; D006801:Humans; D020935:MAP Kinase Signaling System; D010392:Pemphigus / Q000150:complications*; D048051:p38 Mitogen-Activated Protein Kinases / Q000378:metabolism*
chemical_list: D001323:Autoantibodies; C495297:DSG1 protein, human; C071894:DSG3 protein, human; D051183:Desmoglein 1; D051184:Desmoglein 3; D048051:p38 Mitogen-Activated Protein Kinases
keywords: acantholysis; desmoglein; p38 MAPK; pathomechanism; pemphigus