Article
Amelioration of epidermolysis bullosa by transfer of wild-type bone marrow cells.
Blood - 29 Jan 2009
Tolar Jakub, Ishida-Yamamoto Akemi, Riddle Megan, McElmurry Ron T, Osborn Mark, Xia Lily, Lund Troy, Slattery Catherine, Uitto Jouni, Christiano Angela M, Wagner John E, Blazar Bruce R
Abstract excerpt
The recessive dystrophic form of epidermolysis bullosa (RDEB) is a disorder of incurable skin fragility and blistering caused by mutations in the type VII collagen gene (Col7a1). The absence of type VII collagen production leads to the loss of adhesion at the basement membrane zone due to the absence of anchoring fibrils, which are composed of type VII collagen. We report that wild-type, congenic bone marrow...
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