Injury- and inflammation-driven skin fibrosis: The paradigm of epidermolysis bullosa.
作者： Alexander Nyström , Leena Bruckner-Tuderman
DOI: 10.1016/J.MATBIO.2018.01.016
关键词:
摘要: Genetic or acquired destabilization of the dermal extracellular matrix evokes injury- and inflammation-driven progressive soft tissue fibrosis. Dystrophic epidermolysis bullosa (DEB), a heritable human skin fragility disorder, is paradigmatic disease to investigate these processes. Studies DEB have generated abundant new information on cellular molecular mechanisms at play in fibrosis which are not only limited intractable diseases, but also applicable some most common conditions. Here, we discuss recent advances understanding biological mechanical driving DEB. Much this progress owed implementation cell omics studies, pay special attention to. Based novel findings increased DEB, translational aspects future therapeutic perspectives emerging.
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