In general, fibulin-1 is increased in IPF, correlates with disease progression,120 and plays a role in pulmonary fibrogenesis in vivo.121 On a functional level, fibulin-1c seems to activate latent TGFβ by interacting with the LTBP1, thereby facilitating myofibroblast activation and ECM deposition via increased mothers against decapentaplegic homolog 3 (SMAD3) signaling.121. This evidence concerns the gene TGFB1 and idiopathic pulmonary fibrosis.