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Injection of genetically engineered fibroblasts corrects regenerated human epidermolysis bullosa skin tissue JOURNAL OF CLINICAL INVESTIGATION Ortiz-Urda, S., Lin, Q., Green, C. L., Keene, D. R., Marinkovich, M. P., Khavari, P. A. 2003; 111 (2): 251-255

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. This article was published online in advance of the print edition. The date of publication is available from the JCI website, http://www.jci.org.

View details for DOI 10.1172/JCI.200317193

View details for Web of Science ID 000180672900016

View details for PubMedID 12531881

View details for PubMedCentralID PMC151880