Cystic fibrosis revisited

Cystic fibrosis is a pleiotropic disease whose primary defect is thought to be abnormal chloride conductance. Despite intensive study, the role of the protein in the airway and the mechanism for its direct participation in th e disease pathology remain unclear. This paper reviews CFTR's cell regulato ry functions and data supporting the role of CFTR in secretory epithelial c ell development. A hypothesis for CF pathophysiology based on secretory cel l differentiation is proposed. (C) 2000 Academic Press.