Evidence for Periciliary Liquid Layer Depletion, Not Abnormal Ion Composition, in the Pathogenesis of Cystic Fibrosis Airways Disease

@article{Matsui1998EvidenceFP,
  title={Evidence for Periciliary Liquid Layer Depletion, Not Abnormal Ion Composition, in the Pathogenesis of Cystic Fibrosis Airways Disease},
  author={Hirotoshi Matsui and Barbara R. Grubb and Robert Tarran and Scott H. Randell and John T. Gatzy and C. Davis and Richard C. Boucher},
  journal={Cell},
  year={1998},
  volume={95},
  pages={1005-1015}
}
The pathogenesis of cystic fibrosis (CF) airways infection is unknown. Two hypotheses, "hypotonic [low salt]/defensin" and "isotonic volume transport/mucus clearance," attempt to link defects in cystic fibrosis transmembrane conductance regulator-mediated ion transport to CF airways disease. We tested these hypotheses with planar and cylindrical culture models and found no evidence that the liquids lining airway surfaces were hypotonic or that salt concentrations differed between CF and normal… CONTINUE READING
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