Susana Ortiz-Urda, Qun Lin, Cheryl L. Green, Douglas R. Keene, M. Peter Marinkovich, Paul A. Khavari
J Clin Invest.
2003;
111(2):251–255
doi:10.1172/JCI17193
This article Copyright © 2003, The American Society for Clinical Investigation
Abstract
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C
urrent therapeutic strategies for genetic skin disorders rely on the complex process of grafting genetically engineered tissue to recipient wound beds. Because fibroblasts synthesize and secrete extracellular matrix, we explored their utility in recessive dystrophic epidermolysis bullosa (RDEB), a blistering disease due to defective extracellular type VII collagen. Intradermal injection of RDEB fibroblasts overexpressing type VII collagen into intact RDEB skin stably restored correctly localized type VII collagen expression in vivo and normalized hallmark RDEB disease features, including subepidermal blistering and anchoring fibril defects.
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