Airway surface liquid volume regulation determines different airway phenotypes in liddle compared with betaENaC-overexpressing mice.

@article{Mall2010AirwaySL,
  title={Airway surface liquid volume regulation determines different airway phenotypes in liddle compared with betaENaC-overexpressing mice.},
  author={Marcus A Mall and Brian Button and Bjarki J{\'o}hannesson and Zhe Zhou and Alessandra Livraghi and Ray A. Caldwell and Susanne C Schubert and Carsten Schultz and Wanda K. O'Neal and Sylvain Pradervand and Edith Hummler and Bernard C Rossier and Barbara R. Grubb and Richard C. Boucher},
  journal={The Journal of biological chemistry},
  year={2010},
  volume={285 35},
  pages={26945-55}
}
Studies in cystic fibrosis patients and mice overexpressing the epithelial Na(+) channel beta-subunit (betaENaC-Tg) suggest that raised airway Na(+) transport and airway surface liquid (ASL) depletion are central to the pathogenesis of cystic fibrosis lung disease. However, patients or mice with Liddle gain-of-function betaENaC mutations exhibit hypertension but no lung disease. To investigate this apparent paradox, we compared the airway phenotype (nasal versus tracheal) of Liddle with CFTR… CONTINUE READING