Cystic fibrosis (CF) is an autosomal recessive defect occurring in approximately one in 3,500 live births based on data from neonatal screening. CF is clinically characterised by chronic sinopulmonary and GI manifestations, which are caused by abnormalities in the cystic fibrosis transmembrane conductance regulator (CFTR), a channel, located at the surface of the cells lining the airway epithelium and in the submucosal glands that mediate cyclic adenosine monophosphate (cAMP)-regulated transport of chloride and other anions.