In vivo activation of the cystic fibrosis transmembrane conductance regulator mutant deltaF508 in murine nasal epithelium.

@article{Kelley1997InVA,
  title={In vivo activation of the cystic fibrosis transmembrane conductance regulator mutant deltaF508 in murine nasal epithelium.},
  author={Thomas Kelley and Kelly B Thomas and Laura J H Milgram and Mitchell L. Drumm},
  journal={Proceedings of the National Academy of Sciences of the United States of America},
  year={1997},
  volume={94 6},
  pages={2604-8}
}
The gene causing cystic fibrosis (CF) encodes the CF transmembrane conductance regulator (CFTR), a cAMP-regulated chloride channel. Mutations in this gene result in reduced transepithelial chloride permeability across tissues affected in CF. Consequently, restoring chloride permeability to these tissues may prove therapeutic. Here we report that a combination of forskolin, an adenylate cyclase activator, and milrinone, an inhibitor of class III phosphodiesterases, increases the magnitude of the… CONTINUE READING

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