This model will offer new opportunities to investigate the ramifications of CF disease progression, severity, and respiratory pathology resulting from CFTR nonsense mutations in a longitudinal manner (Birket et al., 2018), as well as provide a relevant animal model to study emerging therapies where the mucus defect (Birket et al., 2018) or the pharmacology of CFTR (Birket et al., 2016) are important parameters. Here, CFTR is linked to cystic fibrosis.