Article
Keratinocyte-/fibroblast-targeted rescue of Col7a1-disrupted mice and generation of an exact dystrophic epidermolysis bullosa model using a human COL7A1 mutation.
The American journal of pathology - 1 Dec 2009
Ito Kei, Sawamura Daisuke, Goto Maki, Nakamura Hideki, Nishie Wataru, Sakai Kaori, Natsuga Ken, Shinkuma Satoru, Shibaki Akihiko, Uitto Jouni, Denton Christopher P, Nakajima Osamu, Akiyama Masashi, Shimizu Hiroshi
Abstract excerpt
Recessive dystrophic epidermolysis bullosa (RDEB) is a severe hereditary bullous disease caused by mutations in COL7A1, which encodes type VII collagen (COL7). Col7a1 knockout mice (COL7(m-/-)) exhibit a severe RDEB phenotype and die within a few days after birth. Toward developing novel approaches for treating patients with RDEB, we attempted to rescue COL7(m-/-) mice by introducing human COL7A1 cDNA. We first...
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