Identification of periplakin as a major regulator of lung injury and repair in mice
作者： Valérie Besnard , Rania Dagher , Tania Madjer , Audrey Joannes , Madeleine Jaillet
DOI: 10.1172/JCI.INSIGHT.90163
关键词:
摘要: Periplakin is a component of the desmosomes that acts as cytolinker between intermediate filament scaffolding and desmosomal plaque. strongly expressed by epithelial cells in lung target antigen for autoimmunity idiopathic pulmonary fibrosis. The aim this study was to determine role periplakin during injury remodeling mouse model fibrosis induced bleomycin. We found expression downregulated whole alveolar following bleomycin-induced injury. Deletion Ppl gene mice improved survival reduced development after Notably, deletion promoted an antiinflammatory environment linked profound changes type 2 cells, including overexpression cytokines, decreased profibrotic mediators, altered cell signaling with response TGF-β1. These results identify previously unidentified regulator lung.
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