A mouse model for IPF research

Duerr, J. et al. Nat. Commun. 11, 2012 (2020)

Idiopathic pulmonary fibrosis (IPF) is a progressive lung disease of unknown etiology and with limited therapeutic options. Over the years, progress in IPF research has been hindered by a lack of animal models that recapitulate the progressive evolution of IPF in patients.

A study now shows that conditional deletion of Nedd4-2—a gene encoding E3 ubiquitin-protein ligase NEDD4-like (NEDD4-2), a protein involved in epithelial homeostasis—in lung epithelial cells of mice at adult ages results in a spontaneous and progressive lung disease that shares important features with IPF, including progressive pulmonary fibrosis. The new genetic model provides new insights into IPF pathophysiology and could be an important resource for the identification of biomarkers and therapeutic targets.