It is well-established that the WNT signalling pathway is reactivated in IPF [119,121] and expression of WNT ligands (WNT1, WNT3a), intracellular downstream inducers (β-catenin, GSK-3β), as well as extracellular inhibitors of canonical WNT signalling (Dickkopf proteins DKK1, DKK4 and the interacting transmembrane receptor Kremen 1) has been demonstrated in bronchial and bronchiolar epithelium in IPF [122,123]. Here, DKK1 is linked to idiopathic pulmonary fibrosis.