Journal article
Expression of cystic fibrosis transmembrane conductance regulator corrects defective chloride channel regulation in cystic fibrosis airway epithelial cells
Nature (London), Vol.347(6291), pp.358-363
09/1990
DOI: 10.1038/347358a0
PMID: 1699126
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.
Details
- Title: Subtitle
- Expression of cystic fibrosis transmembrane conductance regulator corrects defective chloride channel regulation in cystic fibrosis airway epithelial cells
- Creators
- Devra P RichMatthew P AndersonRichard J GregorySeng H ChengSucharita PaulDouglas M JeffersonJohn D McCannKatherine W KlingerAlan E SmithMichael J Welsh
- Resource Type
- Journal article
- Publication Details
- Nature (London), Vol.347(6291), pp.358-363
- DOI
- 10.1038/347358a0
- PMID
- 1699126
- ISSN
- 0028-0836
- eISSN
- 1476-4687
- Language
- English
- Date published
- 09/1990
- Academic Unit
- Neurology; Molecular Physiology and Biophysics; Neurosurgery; Internal Medicine
- Record Identifier
- 9984019480402771
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