By conditional deletion of Nedd4-2 (conditional Nedd4-2−/−), we previously generated a mouse model that develops spontaneous, chronically progressive lung disease that recapitulates key features of IPF in patients such as a distinct fibrotic pattern in histology and micro-computed tomography (micro-CT) including honeycombing-like lesions and fibroblast foci-like fibrotic consolidations, resulting in restrictive lung disease in pulmonary function testing and high pulmonary mortality from 3 mo after induction onward (17). Here, NEDD4L is linked to Restrictive ventilatory defect.