When inactive, TGF-β is bound to latency-associated peptide and tethered to the ECM.158, 161, 162, 163Multiple integrins, including αvβ6 on epithelial cells and αvβ1 on fibroblasts, release TGF-β from its inactive state.158, 161, 162αvβ6 protein expression is increased in IPF tissue compared to healthy controls, localizes to epithelial cells overlying fibrotic areas and to areas with active inflammation, and has been associated with increased mortality.164, 165Targeting αvβ6 has been a promising option to treat pulmonary fibrosis. This evidence concerns the gene TGFB1 and idiopathic pulmonary fibrosis.