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Increased airway epithelial Na+ absorption produces cystic fibrosis-like lung disease in mice.


Mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene result in defective epithelial cAMP-dependent Cl(-) secretion and increased airway Na(+) absorption. The mechanistic links between these altered ion transport processes and the pathogenesis of cystic fibrosis lung disease, however, are unclear. To test the hypothesis that accelerated Na(+) transport alone can produce cystic fibrosis-like lung disease, we generated mice with airway-specific overexpression of epithelial Na(+) channels (ENaC). Here we show that increased airway Na(+) absorption in vivo caused airway surface liquid (ASL) volume depletion, increased mucus concentration, delayed mucus transport and mucus adhesion to airway surfaces. Defective mucus transport caused a severe spontaneous lung disease sharing features with cystic fibrosis, including mucus obstruction, goblet cell metaplasia, neutrophilic inflammation and poor bacterial clearance. We conclude that increasing airway Na(+) absorption initiates cystic fibrosis-like lung disease and produces a model for the study of the pathogenesis and therapy of this disease.

Pubmed ID: 15077107


  • Mall M
  • Grubb BR
  • Harkema JR
  • O'Neal WK
  • Boucher RC


Nature medicine

Publication Data

May 3, 2004

Associated Grants


Mesh Terms

  • Animals
  • Cystic Fibrosis
  • Cystic Fibrosis Transmembrane Conductance Regulator
  • Disease Models, Animal
  • Epithelial Sodium Channels
  • Epithelium
  • Humans
  • Lung
  • Mice
  • Mice, Inbred C3H
  • Mice, Inbred C57BL
  • Mice, Transgenic
  • Mucus
  • Sodium
  • Sodium Channels