These studies also identified the dysregulation of (i) ENaC, leading to airway surface liquid depletion and reduced mucociliary clearance; (ii) proSP-C biogenesis and (iii) TGFβ/Smad signaling, promoting fibrotic remodeling as epithelial defects and potential mechanisms triggering IPF-like disease in adult conditional Nedd4-2−/− mice [9]. Here, NEDD4L is linked to idiopathic pulmonary fibrosis.