Injection of genetically engineered fibroblasts corrects regenerated human epidermolysis bullosa skin tissue
Susana Ortiz-Urda, … , M. Peter Marinkovich, Paul A. Khavari
Susana Ortiz-Urda, … , M. Peter Marinkovich, Paul A. Khavari
Published January 15, 2003
Citation Information: J Clin Invest. 2003;111(2):251-255. https://doi.org/10.1172/JCI17193.
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Article Dermatology

Injection of genetically engineered fibroblasts corrects regenerated human epidermolysis bullosa skin tissue

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.

Authors

Susana Ortiz-Urda, Qun Lin, Cheryl L. Green, Douglas R. Keene, M. Peter Marinkovich, Paul A. Khavari

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Figure 4

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Restoration of anchoring fibrils after intradermal fibroblast injection....
Restoration of anchoring fibrils after intradermal fibroblast injection. BMZ ultrastructure of human skin tissue regenerated on CB.17 scid/scid mice. Note the absence of anchoring fibrils in RDEB skin injected with RDEB– fibroblasts (middle panel) and their restoration in RDEB skin injected with RDEB+ fibroblasts (right panel). Normal skin control (left panel) was produced using cells from normal subjects. Arrows denote representative anchoring fibrils. Scale bar: 500 nm. ld, lamina densa.