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...

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Bibliographic Details
Published in:Nature (London) 1990-09, Vol.347 (6291), p.358-363
Main Authors: Rich, Devra P, Anderson, Matthew P, Gregory, Richard J, Cheng, Seng H, Paul, Sucharita, Jefferson, Douglas M, McCann, John D, Klinger, Katherine W, Smith, Alan E, Welsh, Michael J
Format: Article
Language:English
Subjects:
Biological and medical sciences
Cell Membrane Permeability
Cell membranes
Cells, Cultured
chloride
Chloride Channels
Chloride conductance
Chloride ions
Chlorides - metabolism
Cyclic AMP - pharmacology
Cystic fibrosis
Cystic Fibrosis - genetics
Cystic Fibrosis - metabolism
Cystic Fibrosis Transmembrane Conductance Regulator
Cytosol
Dialysis
Electric Conductivity
Epithelial cells
Epithelium - drug effects
Epithelium - metabolism
Fluorescence
Fundamental and applied biological sciences. Psychology
Gene Expression
Humanities and Social Sciences
Humans
Ion channels
Kinases
Kinetics
Lymphocytes B
Membrane Proteins - genetics
Membrane Proteins - metabolism
Membrane Proteins - physiology
Microscopy, Fluorescence
Molecular and cellular biology
Molecular genetics
multidisciplinary
Mutation
Nystatin
Permeability
Proteins
Respiratory System - metabolism
Respiratory tract
RNA polymerase
Science
Transfection
transmembrane conductance regulator
Vaccinia virus - genetics
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Description
Summary: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.
ISSN:0028-0836
1476-4687
DOI:10.1038/347358a0