Article
Expression of cystic fibrosis transmembrane conductance regulator corrects defective chloride channel regulation in cystic fibrosis airway epithelial cells.
Nature - 27 Sept 1990
Rich D P, Anderson M P, Gregory R J, Cheng S H, Paul S, Jefferson D M, McCann J D, Klinger K W, Smith A E, Welsh M J
Abstract excerpt
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 (delta F508), corrected the Cl- channel defect. Correction of the phenotypic defect demonstrates a causal...
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