Role of airway surface liquid and submucosal glands in cystic fibrosis lung disease.

@article{Verkman2003RoleOA,
  title={Role of airway surface liquid and submucosal glands in cystic fibrosis lung disease.},
  author={Alan S. Verkman and Yuanlin Song and Jay R. Thiagarajah},
  journal={American journal of physiology. Cell physiology},
  year={2003},
  volume={284 1},
  pages={
          C2-15
        }
}
Cystic fibrosis (CF) is caused by mutations in the CF transmembrane conductance regulator (CFTR) protein, an epithelial chloride channel expressed in the airways, pancreas, testis, and other tissues. A central question is how defective CFTR function in CF leads to chronic lung infection and deterioration of lung function. Several mechanisms have been proposed to explain lung disease in CF, including abnormal airway surface liquid (ASL) properties, defective airway submucosal gland function… 
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Cystic fibrosis and airway submucosal glands

The demonstration that the predominant sites of expression of the CF gene in normal lungs are the submucosal glands, together with the histological observations showing that hyperplasia of these glands and mucin occlusion of the gland ducts are the earliest signs of disease in the CF lung, suggest that malfunction of the submunity glands may be an important factor contributing to the early pathophysiology of CF lung disease.

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...

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