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Expression of cystic fibrosis transmembrane conductance regulator corrects defective chloride channel regulation in cystic fibrosis airway epithelial cells

Nature volume 347, pages 358–363 (1990)Cite this article

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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Author information

Affiliations

  1. Howard Hughes Medical Institute, Departments of Internal Medicine and Physiology and Biophysics, University of Iowa College of Medicine, Iowa City, Iowa, 52242, USA

    Devra P. Rich, Matthew P. Anderson & John D. McCann

  2. Genzyme Corporation, One Mountain Road, Framingham, Massachusetts, 01701, USA

    Richard J. Gregory, Seng H. Cheng, Sucharita Paul & Alan E. Smith

  3. IG Laboratories Inc., One Mountain Road, Framingham, Massachusetts, 01701, USA

    Katherine W. Klinger

  4. Department of Physiology, Tufts University School of Medicine, and Departments of Pediatrics and Medicine, New England Medical Center, Boston, Massachusetts, 02111, USA

    Douglas M. Jefferson

Authors
  1. Devra P. Rich
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  2. Matthew P. Anderson
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  3. Richard J. Gregory
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  4. Seng H. Cheng
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  5. Sucharita Paul
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  6. Douglas M. Jefferson
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  7. John D. McCann
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  8. Katherine W. Klinger
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  9. Alan E. Smith
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  10. Michael J. Welsh
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Rich, D., Anderson, M., Gregory, R. et al. Expression of cystic fibrosis transmembrane conductance regulator corrects defective chloride channel regulation in cystic fibrosis airway epithelial cells. Nature 347, 358–363 (1990). https://doi.org/10.1038/347358a0

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  • DOI: https://doi.org/10.1038/347358a0

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