NEDD4L and cystic fibrosis: Our study showed normal lung structures with preserved alveolar pattern and without any respiratory distress during lifetime (Fig. 8b), whereas recent studies using lung epithelial Nedd4-2 KO mice revealed lung disease associated with cystic fibrosis-like changes18 and earlier mice Nedd4-2 KO mice to target terminal exons of mouse Nedd4-2 resulted in lethal respiratory distress during the perinatal period7.