Research Techniques Made Simple: Mouse Models of Autoimmune Blistering Diseases

详细信息    查看全文

• 作者：Robert Pollmann¹ ; Rü ; diger Eming¹ ; ^{eming@med.uni-marburg.de}
• 关键词：AIDB ; autoimmune blistering disease ; BP ; bullous pemphigoid ; COL-17 ; type XVII collagen ; COL-7 ; type VII collagen ; DH ; dermatitis herpetiformis ; EBA ; epidermolysis bullosa acquisita ; MHC ; major histocompatibility complex ; PV ; pemphigus vulgaris
• 刊名：Journal of Investigative Dermatology
• 出版年：2017
• 出版时间：January 2017
• 年：2017
• 卷：137
• 期：1
• 页码：e1-e6
• 全文大小：1127 K
• 卷排序：137

文摘

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.