Molecular Therapy

FIGURE 2

FROM:

Injection of Recombinant Human Type VII Collagen Corrects the Disease Phenotype in a Murine Model of Dystrophic Epidermolysis Bullosa

Jennifer Remington, Xinyi Wang, Yingpin Hou, Hui Zhou, Julie Burnett, Trevor Muirhead, Jouni Uitto, Douglas R Keene, David T Woodley and Mei Chen

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Figure 2.

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Presence of type VII collagen (C7) in dystrophic epidermolysis bullosa (DEB) mice following protein injection. (a) DEB mice were intradermally injected on their dorsal surface with 10 microg C7 once every day for 4 consecutive days, and tissue sections obtained from skin at various distances from the injection site 1 week after injections were subjected to immunostaining using an anti-NC1 antibody. Panels A, B, and C are biopsies taken from the immediate injected area, near by and far away, respectively. Note that the injected human C7 migrated from the dermis and incorporated into the DEB mouse basement membrane zone at the injected dorsal side. Interestingly, some biopsies obtained from sites remote from the injection sites, such as neck (panel D), abdomen (panel E), and paw (panel F), also revealed C7 staining. (b) Dorsal side of skin from DEB mice injected intradermally with 10 microg of purified recombinant human C7 once every day for the first week and then weekly thereafter was stained with an anti-NC1 antibody. Panels A, B, and C are biopsies taken 6 hours, 15 weeks, or 28 weeks, respectively. For panel D, DEB mice (n = 10) were injected with 60 microg C7 for the first week only, and tissue sections obtained from the skin at 8 weeks after initial injections were subjected to immunostaining using an anti-NC1 antibody. e, epidermis; d, dermis.

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