Article
Diversity of Mechanisms Underlying Latent TGF-β Activation in Recessive Dystrophic Epidermolysis Bullosa.
The Journal of investigative dermatology - 1 Jun 2021
Akasaka Eijiro, Kleiser Svenja, Sengle Gerhard, Bruckner-Tuderman Leena, Nyström Alexander
Abstract excerpt
Injury- and inflammation-driven progressive dermal fibrosis is a severe manifestation of recessive dystrophic epidermolysis bullosa-a genetic skin blistering disease caused by mutations in COL7A1. TGF-β activation plays a prominent part in progressing dermal fibrosis. However, the underlying mechanisms are not fully elucidated. TGF-β is secreted in a latent form, which has to be activated for its biological...
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