TGFB1 and pulmonary fibrosis: Further, at the whole organ level, our proteomics analyses show that in vivo treatment with pirfenidone reverted major proteomic changes associated with pulmonary fibrosis including dysregulation of TGFβ-induced ECM proteins in conditional Nedd4-2−/− mice (Fig. 8, Fig. 9; Supplementary Fig. 5).