Article
A cell-based drug discovery assay identifies inhibition of cell stress responses as a new approach to treatment of epidermolysis bullosa simplex.
Journal of cell science - 1 Oct 2021
Tan Tong San, Common John E A, Lim John S Y, Badowski Cedric, Firdaus Muhammad Jasrie, Leonardi Steven S, Lane E Birgitte
Abstract excerpt
In the skin fragility disorder epidermolysis bullosa simplex (EBS), mutations in keratin 14 (K14, also known as KRT14) or keratin 5 (K5, also known as KRT5) lead to keratinocyte rupture and skin blistering. Severe forms of EBS are associated with cytoplasmic protein aggregates, with elevated kinase activation of ERK1 and ERK2 (ERK1/2; also known as MAPK3 and MAPK1, respectively), suggesting intrinsic stress...
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