title: Research Techniques Made Simple: Mouse Models of Autoimmune Blistering Diseases.
abstract: Autoimmune blistering diseases are examples of autoantibody-mediated, organ-specific autoimmune disorders. Based on a genetic susceptibility, such as a strong HLA-class II association, as yet unknown triggering factors induce the formation of circulating and tissue-bound autoantibodies that are mainly directed against adhesion structures of the skin and mucous membranes. Compared with other autoimmune diseases, especially systemic disorders, the pathogenicity of autoimmune blistering diseases is relatively well described. Several animal models of autoimmune blistering diseases have been established that helped to uncover the immunological and molecular mechanisms underlying the blistering phenotypes. Each in vivo model focuses on specific aspects of the autoimmune cascade, from loss of immunological tolerance on the level of T and B cells to the pathogenic effects of autoantibodies upon binding to their target autoantigen. We discuss current mouse models of autoimmune blistering diseases, including models of pemphigus vulgaris, bullous pemphigoid, epidermolysis bullosa acquisita, and dermatitis herpetiformis.
authors.affiliation: Department of Dermatology and Allergology, Philipps University Marburg, Marburg, Germany.
authors.forename: Robert
authors.initials: R
authors.lastname: Pollmann
authors.affiliation: Department of Dermatology and Allergology, Philipps University Marburg, Marburg, Germany. Electronic address: eming@med.uni-marburg.de.
authors.forename: Rüdiger
authors.initials: R
authors.lastname: Eming
mesh_terms: D000818:Animals; D001324:Autoantigens / Q000276:immunology*; D001327:Autoimmune Diseases / Q000276:immunology* / Q000473:pathology; D001707:Biopsy, Needle; D003874:Dermatitis Herpetiformis / Q000276:immunology / Q000473:pathology; D004195:Disease Models, Animal; D016107:Epidermolysis Bullosa Acquisita / Q000276:immunology / Q000473:pathology; D005260:Female; D007150:Immunohistochemistry; D051379:Mice; D008810:Mice, Inbred C57BL; D008822:Mice, Transgenic; D010391:Pemphigoid, Bullous / Q000276:immunology / Q000473:pathology; D010392:Pemphigus / Q000276:immunology / Q000473:pathology; D012107:Research Design; D012872:Skin Diseases, Vesiculobullous / Q000276:immunology* / Q000473:pathology
chemical_list: D001324:Autoantigens