EXPRESSION OF CYSTIC-FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR CORRECTS DEFECTIVE CHLORIDE CHANNEL REGULATION IN CYSTIC-FIBROSIS AIRWAY EPITHELIAL-CELLS

The cystic fibrosis transmembrane conductance regulator (CFTR) was expressed in cultured cystic fibrosis airway epithelial cells and Cl- channel activation assessed in single cells using a fluorescence microscopic assay and the patch-clamp technique. Expression of CFTR, but not of a mutant form of CFTR (ΔF508), corrected the Cl- channel defect. Correction of the phenotypic defect demonstrates a causal relationship between mutations in the CFTR gene and defective Cl- transport which is the hallmark of the disease. © 1990 Nature Publishing Group.