Ion Transport in Normal and CF Airway Epithelia
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.
KeywordsCystic Fibrosis Apical Membrane Airway Epithelium Cystic Fibrosis Transmembrane Regulator Airway Surface Liquid
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