Injection of genetically engineered fibroblasts corrects regenerated human epidermolysis bullosa skin tissue
Open Access
- 15 January 2003
- journal article
- Published by American Society for Clinical Investigation in Journal of Clinical Investigation
- Vol. 111 (2) , 251-255
- https://doi.org/10.1172/jci200317193
Abstract
Current 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.Keywords
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