Defective fluid transport by cystic fibrosis airway epithelia.

@article{Smith1994DefectiveFT,
  title={Defective fluid transport by cystic fibrosis airway epithelia.},
  author={Jeffrey John Smith and Philip H. Karp and Michael J Welsh},
  journal={The Journal of clinical investigation},
  year={1994},
  volume={93 3},
  pages={
          1307-11
        }
}
Cystic fibrosis (CF) airway epithelia exhibit defective transepithelial electrolyte transport: cAMP-stimulated Cl- secretion is abolished because of the loss of apical membrane cystic fibrosis transmembrane conductance regulator (CFTR) Cl- channels, and amiloride-sensitive Na+ absorption is increased two- to threefold because of increased amiloride-sensitive apical Na+ permeability. These abnormalities are thought to alter respiratory tract fluid, thereby contributing to airway disease, the… CONTINUE READING
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