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Role of airway surface liquid and submucosal glands in cystic fibrosis lung disease
- Source :
- American Journal of Physiology-Cell Physiology. 284:C2-C15
- Publication Year :
- 2003
- Publisher :
- American Physiological Society, 2003.
-
Abstract
- 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, altered inflammatory response, defective organellar acidification, loss of CFTR regulation of plasma membrane ion transporters, and others. This review focuses on the physiology of the ASL and submucosal glands with regard to their proposed role in CF lung disease. Experimental evidence for defective ASL properties and gland function in CF is reviewed, and deficiencies in understanding ASL/gland physiology are identified as areas for further investigation. New model systems and measurement technologies are being developed to make progress in establishing lung disease mechanisms in CF, which should facilitate mechanism-based design of therapies for CF.
- Subjects :
- Exocrine gland
Pathology
medicine.medical_specialty
Pancreatic disease
Cystic Fibrosis
Surface Properties
Physiology
Cystic Fibrosis Transmembrane Conductance Regulator
Respiratory Mucosa
Biology
Cystic fibrosis
Exocrine Glands
medicine
Animals
Humans
Lung
Submucosal glands
Respiratory disease
Cell Biology
respiratory system
medicine.disease
Cystic fibrosis transmembrane conductance regulator
respiratory tract diseases
medicine.anatomical_structure
Chloride channel
biology.protein
Subjects
Details
- ISSN :
- 15221563 and 03636143
- Volume :
- 284
- Database :
- OpenAIRE
- Journal :
- American Journal of Physiology-Cell Physiology
- Accession number :
- edsair.doi.dedup.....3dd59fff0eaee66c0367744f6cbbb851