Article
A hypomorphic mouse model of dystrophic epidermolysis bullosa reveals mechanisms of disease and response to fibroblast therapy.
The Journal of clinical investigation - 1 May 2008
Fritsch Anja, Loeckermann Stefan, Kern Johannes S, Braun Attila, Bösl Michael R, Bley Thorsten A, Schumann Hauke, von Elverfeldt Dominik, Paul Dominik, Erlacher Miriam, Berens von Rautenfeld Dirk, Hausser Ingrid, Fässler Reinhard, Bruckner-Tuderman Leena
Abstract excerpt
Dystrophic epidermolysis bullosa (DEB) is a severe skin fragility disorder associated with trauma-induced blistering, progressive soft tissue scarring, and increased risk of skin cancer. DEB is caused by mutations in type VII collagen. In this study, we describe the generation of a collagen VII hypomorphic mouse that serves as an immunocompetent animal model for DEB. These mice expressed collagen VII at about 10%...
Read the complete abstract on PubMedTopics
Share this publication in a Topic to start or enrich a Post.