Nature Communications (Apr 2020)

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

  • 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

DOI
https://doi.org/10.1038/s41467-020-15743-6
Journal volume & issue
Vol. 11, no. 1
pp. 1 – 18

Abstract

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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.