Subsequent studies in mice with constitutive lung-specific deletion of Nedd4-2 using a “leaky” Nedd4-2fl/fl/Sftpc-rtTA/Cre triple transgenic system under the control of the surfactant protein C (Sftpc) promoter showed massive neutrophilic inflammation, aspects of cystic fibrosis-like lung disease and premature death 3–4 weeks after birth [11]. This evidence concerns the gene NEDD4L and cystic fibrosis.