Article
Kinase Inhibition by PKC412 Prevents Epithelial Sheet Damage in Autosomal Dominant Epidermolysis Bullosa Simplex through Keratin and Cell Contact Stabilization.
The Journal of investigative dermatology - 1 Dec 2022
Rietscher Katrin, Jahnke Heinz-Georg, Rübsam Matthias, Lin Eric W, Has Cristina, Omary M Bishr, Niessen Carien M, Magin Thomas M
Abstract excerpt
Epidermolysis bullosa simplex (EBS) is a severe and potentially life-threatening disorder for which no adequate therapy exists. Most cases are caused by dominant sequence variations in keratin genes K5 or K14, leading to the formation of cytoplasmic keratin aggregates, profound keratinocyte fragility, and cytolysis. We hypothesized that pharmacological reduction of keratin aggregates, which compromise...
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