Nedd4-2 deficiency has been shown to trigger fibrotic remodeling within the lung parenchyma by chronic injury of the epithelium, among others due to reduced mucociliary clearance, a mechanism which has been suggested to be of relevance for formation of microscopic honeycomb cysts in IPF [15,28]. This evidence concerns the gene NEDD4L and idiopathic pulmonary fibrosis.