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

Duerr J, Leitz DHW, Szczygiel M, Dvornikov D, Fraumann SG, Kreutz C, Zadora PK, Seyhan Agircan A, Konietzke P, Engelmann TA, Hegermann J, Mulugeta S, Kawabe H, Knudsen L, Ochs M, Rotin D, Muley T, Kreuter M, Herth FJF, Wielpütz MO, Beers MF, Klingmüller U, Mall MA.

Nat Commun. 2020;11(1):2012

Nat Commun.                online article

It is shown that conditional deletion of Nedd4-2 in lung epithelial cells in adult mice produces chronic lung disease sharing key features with Idiopathic pulmonary fibrosis (IPF). This model may therefore aid studies about the pathogenesis and therapy of IPF.