Molecular Therapy

FIGURE 3

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 3.

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Absence of type VII collagen (C7) in blood stream and internal organs. (a) Sera were taken from either control wild-type mice (WT) without injection or dystrophic epidermolysis bullosa (DEB) mice that were injected with C7 once every day for the first week and then weekly thereafter at the time indicated and subjected to 6% sodium dodecyl sulfate polyacrylamide sulfate followed by immunoblot analysis using an anti-NC1 antibody. One hundred and fifty nanograms of purified recombinant C7 was run as a control (Con). The positions of full-length 290-kd C7, 70-kd mouse IgG heavy chain (mIgGH), and molecular weight markers are indicated. (b) Tissue distribution of intradermally injected C7. Four weeks after injection of C7, necropsies were performed on the DEB mice (n = 6), and tissue sections obtained from brain, kidney, liver, lung, spleen, heart, small intestine (SI), and skin were subjected to immunostaining using an anti-NC1 antibody. Note that the injected C7 was readily detected in the skin, but not in any other organs.

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