Article
Induction of inflammatory cytokines by a keratin mutation and their repression by a small molecule in a mouse model for EBS.
The Journal of investigative dermatology - 1 Dec 2007
Lu Hong, Chen Jian, Planko Laura, Zigrino Paola, Klein-Hitpass Ludger, Magin Thomas M
Abstract excerpt
Epidermolysis bullosa simplex (EBS) is a skin disorder caused by mutations in keratin (K) 5 or K14 genes. It is widely regarded as a mechanobullous disease, resulting from a weakened cytoskeleton, causing extensive cytolysis. It was postulated by others that certain K14 mutations induce tumor necrosis factor-alpha (TNF-alpha) and increase apoptosis. Here, we report that in K5-/- mice and in a cell culture model...
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