Taken together, the conditional Nedd4-2−/− mice are distinct from the mouse model of BILF demonstrated by a greater similarity to IPF in patients, which has previously also been shown at the histological level with microscopic honeycombing, fibroblast foci-like structures, bronchiolization of peripheral airways, and at the molecular level with altered pulmonary expression of Muc5b and a similarly altered proteomic signature as in IPF (17). The gene discussed is NEDD4L; the disease is idiopathic pulmonary fibrosis.