Conditional deletion of Nedd4-2 in lung epithelial cells causes progressive pulmonary fibrosis in adult mice

Idiopathic pulmonary fibrosis (IPF) is a devastating disease with poor prognosis. Here, the authors show that deficiency of the E3 ubiqutin-protein ligase Nedd4-2 in airway epithelial cells causes IPF-like disease in adult mice. This model may aid studies of the pathogenesis and therapy of IPF.

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Autores principales: Julia Duerr, Dominik H. W. Leitz, Magdalena Szczygiel, Dmytro Dvornikov, Simon G. Fraumann, Clemens Kreutz, Piotr K. Zadora, Ayça Seyhan Agircan, Philip Konietzke, Theresa A. Engelmann, Jan Hegermann, Surafel Mulugeta, Hiroshi Kawabe, Lars Knudsen, Matthias Ochs, Daniela Rotin, Thomas Muley, Michael Kreuter, Felix J. F. Herth, Mark O. Wielpütz, Michael F. Beers, Ursula Klingmüller, Marcus A. Mall
Formato: article
Lenguaje:EN
Publicado: Nature Portfolio 2020
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Acceso en línea:https://doaj.org/article/ae55c88d44b14c0496555b2c7a52b8da
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Sumario:Idiopathic pulmonary fibrosis (IPF) is a devastating disease with poor prognosis. Here, the authors show that deficiency of the E3 ubiqutin-protein ligase Nedd4-2 in airway epithelial cells causes IPF-like disease in adult mice. This model may aid studies of the pathogenesis and therapy of IPF.