Cystic fibrosis (CF) is characterized by a solute transport defect in epithelial tissues. In the
lungs, this defect culminates in the dehydration of the airway surface and mucus
accumulation, ultimately leading to chronic bacterial infection. To date, the current
therapeutic approaches used to treat CF primarily focus on the secondary manifestations of
the disease (eg bacterial infection, viscous mucus). However, new therapeutic approaches
are targeting the underlying ion transport defect in cystic fibrosis, with the aim of restoring the …