Ion Transport Regulation in Cystic Fibrosis Epithelia

Cystic fibrosis (CF) is an autosomal recessive inherited disease that is manifest predominantly among Caucasians. In the Netherlands the average age of survival is 21 years. The clinical symptoms of CF are meconium ileus, pancreatic insufficiency, and/or obstruction of the deeper regions of the airways. In 99% of patients, salt wasting in the sweat is apparent. A single locus appears to be involved in CF, and the defective gene has been located on chromosome 7 (Wainwright et al. 1985).