Collectively, this pattern of dysregulation of ECM proteins is consistent with increased activation of the TGFβ signaling pathway and the similarities between conditional Nedd4-2−/− mice and IPF patients support the relevance of this model for further studies of the complex in vivo pathogenesis of the fibroproliferative response in pulmonary fibrosis. The gene discussed is NEDD4; the disease is idiopathic pulmonary fibrosis.