Injection of genetically engineered fibroblasts corrects regenerated human epidermolysis bullosa skin tissue
J. Clin. Invest. Susana Ortiz-Urda, et al. 111:251 doi:10.1172/JCI17193 [Go to this article.]

Figure 3
Fibroblast-mediated type VII collagen delivery to human RDEB skin. (a) Type VII collagen (green, first column) is shown in human RDEB skin regenerated on immune-deficient mice after intradermal injection with the cell types noted at left. Note the lack of type VII collagen in skin injected with RDEB^– fibroblasts (top row) and its BMZ localization in skin injected with RDEB⁺ fibroblasts (middle row; arrows in dermis denote a collection of intradermal RDEB⁺ cells). Note the blistering seen past the border of human type VII collagen protein (bottom row). The human origin of the skin tissue studied was confirmed using species-specific antibodies to involucrin (orange, second column); triple-stained specimens with serial histological sections are shown. Scale bar: 50 μm. (b) RDEB⁺ fibroblasts in RDEB skin tissue. Note type VII collagen–positive dermal cells (arrows) with typical elongated fibroblast morphology in tissue injected with RDEB⁺ cells and the complete absence of detectable type VII collagen in RDEB skin tissue injected with RDEB^– cells. Scale bars: top panels, 75 μm; bottom panels, 15 μm. E, epidermis; D, dermis; bl, blister.