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Ion Transport in Normal and CF Airway Epithelia

  • Richard C. Boucher
  • Thierry Chinet
  • Niels Willumsen
  • Michael R. Knowles
  • M. Jackson Stutts
Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 290)

Abstract

Cystic fibrosis (CF) of the lung is characterized by chronic suppurative airways infection in which two bacterial species, Pseudomonas aeruginosa and Staphylococcus aureus, predominate. Because no abnormalities in the migratory inflammatory cells or immunoglobulins that protect airway surfaces from infection have been detected in CF, most efforts to analyze the pathophysiology of CF airways disease have focused on elements of the mucociliary transport apparatus. This system is typically analyzed in terms of three components: 1) cilia/cilial activity; 2) mucus glyco-proteins; and, 3) the aqueous, periciliary layer. Most data indicate that cilial motility is normal in CF and cannot account for abnormalities and clearance of secretions. Compositional abnormalities, particularly relating to sulfate ions (1), may exist beween CF and normal mucins but the effects of these abnormalities on mucus clearance are not clear. Because bioelectric abnormalities have been detected routinely in CF airway epithelia, attention has focused on the electrogenic ion transport processes that regulate the depth and composition of the periciliary liquid layer.

Keywords

Cystic Fibrosis Apical Membrane Airway Epithelium Cystic Fibrosis Transmembrane Regulator Airway Surface Liquid 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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Copyright information

© Springer Science+Business Media New York 1991

Authors and Affiliations

  • Richard C. Boucher
    • 1
  • Thierry Chinet
    • 1
  • Niels Willumsen
    • 1
  • Michael R. Knowles
    • 1
  • M. Jackson Stutts
    • 1
  1. 1.Department of Medicine, Division of Pulmonary DiseasesUniversity of North Carolina at Chapel HillChapel HillUSA

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