Biomedicine

Skin Treatments

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Science  04 Oct 2013:
Vol. 342, Issue 6154, pp. 15
DOI: 10.1126/science.342.6154.15-d
CREDIT: A. GOSTYNSKI ET AL., JOURNAL OF INVESTIGATIVE DERMATOLOGY 133 (15 AUGUST 2013) © 2013 THE SOCIETY FOR INVESTIGATIVE DERMATOLOGY

Epidermolysis bullosa is the term used to describe a group of inherited disorders that are characterized by severe blistering and skin fragility. Two recent papers show the beginnings of new therapeutic approaches. Dystrophic epidermolysis bullosa is associated with mutations in type VII collagen that result in blistering, deformities, and aggressive squamous cell carcinoma. Woodley et al. demonstrated that intravenously injected, recombinant type VII collagen was able to home to the region of wounds and restore expression for several weeks in two animal models. Junctional epidermolysis bullosa, in which the causative mutation may occur in the type XVII collagen gene, is corrected in some cells by a secondary mutation, leading to revertant mosaicism. Revertant keratinocytes should have significant potential as an autologous therapeutic agent. Gostynski et al. found that although colony-forming potential was high, revertant cells divided more slowly than wild-type cells. However, revertant cells were capable of engraftment and skin regeneration in a humanized mouse model if the cells were first grown as part of a skin equivalent on a plasma-based scaffold.

J. Invest. Dermatol. 133, 1910; 10.1038/jid.2013.308 (2013).

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