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Mouse Nedd4-2 and control with Pirfenidone

ABSTRACT: Idiopathic pulmonary fibrosis (IPF) is a chronic progressive interstitial lung disease characterized by patchy scarring of the distal lung with limited therapeutic options and poor prognosis. Here, we show that conditional deletion of the ubiquitin ligase Nedd4-2 in lung epithelial cells in adult mice produces chronic lung disease that shares key features with IPF including progressive fibrosis and bronchiolization with increased expression of Muc5b in peripheral airways, honeycombing and characteristic alterations in the lung proteome.

INSTRUMENT(S): Q Exactive

ORGANISM(S): Mus Musculus (mouse)

TISSUE(S): Lung

SUBMITTER: Alexander Held

LAB HEAD: Ursula Klingmüller

PROVIDER: PXD011119 | Pride | 2020-03-20

REPOSITORIES: Pride

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

Duerr Julia J Leitz Dominik H W DHW Szczygiel Magdalena M Dvornikov Dmytro D Fraumann Simon G SG Kreutz Clemens C Zadora Piotr K PK Seyhan Agircan Ayça A Konietzke Philip P Engelmann Theresa A TA Hegermann Jan J Mulugeta Surafel S Kawabe Hiroshi H Knudsen Lars L Ochs Matthias M Rotin Daniela D Muley Thomas T Kreuter Michael M Herth Felix J F FJF Wielpütz Mark O MO Beers Michael F MF Klingmüller Ursula U Mall Marcus A MA

Nature communications 20200424 1

Idiopathic pulmonary fibrosis (IPF) is a chronic progressive interstitial lung disease characterized by patchy scarring of the distal lung with limited therapeutic options and poor prognosis. Here, we show that conditional deletion of the ubiquitin ligase Nedd4-2 (Nedd4l) in lung epithelial cells in adult mice produces chronic lung disease sharing key features with IPF including progressive fibrosis and bronchiolization with increased expression of Muc5b in peripheral airways, honeycombing and c ...[more]

PMID: 32332792

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