Animal model for dystrophic epidermolysis bullosa and acquired epidermolysis bullosa using genetically engineered mice of type VII collagen

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Type VII collagen is a structural protein within basement membrane zone and its gene defect leads to dystrophic epidermolysis bullosa. Also generation of autoantibody against type Vii collagen causes acquired epidermolysis bullosa. Thus, type VII collagen is a major protein in the field of dermatology. The purposes of this study are to establish models of those type VII collagen related diseases and make great contributions for disclosing disease mechanisms and development of new effective treatments using genetically engineered mice. In this study we could generate model mouse of dystrophic epidermolysis which can survive for long time. However, we could not detect auto antibody against type VII collagen in the model mice for acquired epidermolysis. Furthermore, we performed mutation analysis of patients with epidermolysis bullosa.