TGFB1 and idiopathic pulmonary fibrosis: 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.