Expression of cystic fibrosis transmembrane conductance regulator corrects defective chloride channel regulation in cystic fibrosis airway epithelial cells
DP Rich, MP Anderson, RJ Gregory, SH Cheng, S Paul… - Nature, 1990 - nature.com
DP Rich, MP Anderson, RJ Gregory, SH Cheng, S Paul, DM Jefferson, JD McCann…
Nature, 1990•nature.comThe 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.
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.
Abstract
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.
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