Physiol Rev Fuel your research with LabChart
HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS
QUICK SEARCH:   [advanced]


     

Physiol. Rev. 79: 215-255, 1999;
0031-9333/99 $15.00
This Article
Right arrow Full Text
Right arrow Full Text (PDF)
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Right arrow Citation Map
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Alert me to new issues of the journal
Right arrow Download to citation manager
Citing Articles
Right arrow Citing Articles via HighWire
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by PILEWSKI, J. M.
Right arrow Articles by FRIZZELL, R. A.
Right arrow Search for Related Content
PubMed
Right arrow Articles by PILEWSKI, J. M.
Right arrow Articles by FRIZZELL, R. A.

PHYSIOLOGICAL REVIEWS   Vol. 79 No. 1 January 1999, pp. S215-S255
Copyright ©1999 The American Physiological Society

Role of CFTR in Airway Disease

JOSEPH M. PILEWSKI AND RAYMOND A. FRIZZELL

Departments of Medicine and of Cell Biology and Physiology, University of Pittsburgh, Pittsburgh, Pennsylvania

Pilewski, Joseph M., and Raymond A. Frizzell. Role of CFTR in Airway Disease. Physiol. Rev. 79, Suppl.: S215-S255, 1999. --- Cystic fibrosis (CF) is caused by mutations in the gene encoding the CF transmembrane conductance regulator (CFTR), which accounts for the cAMP-regulated chloride conductance of airway epithelial cells. Lung disease is the chief cause of morbidity and mortality in CF patients. This review focuses on mechanisms whereby the deletion or impairment of CFTR chloride channel function produces lung disease. It examines the major themes of the channel hypothesis of CF, which involve impaired regulation of airway surface fluid volume or composition. Available evidence indicates that the effect of CFTR deletion alters physiological functions of both surface and submucosal gland epithelia. At the airway surface, deletion of CFTR causes hyperabsorption of sodium chloride and a reduction in the periciliary salt and water content, which impairs mucociliary clearance. In submucosal glands, loss of CFTR-mediated salt and water secretion compromises the clearance of mucins and a variety of defense substances onto the airway surface. Impaired mucociliary clearance, together with CFTR-related changes in the airway surface microenvironment, leads to a progressive cycle of infection, inflammation, and declining lung function. Here, we provide the details of this pathophysiological cascade in the hope that its understanding will promote the development of new therapies for CF.




This article has been cited by other articles:


Home page
 J. Physiol.Home page
M.-C. Battista, E. Calvo, A. Chorvatova, B. Comte, J. Corbeil, and M. Brochu
Intra-uterine growth restriction and the programming of left ventricular remodelling in female rats
J. Physiol., May 15, 2005; 565(1): 197 - 205.
[Abstract] [Full Text] [PDF]

Home page
 J. Biol. Chem.Home page
K. Taouil, J. Hinnrasky, C. Hologne, P. Corlieu, J.-M. Klossek, and E. Puchelle
Stimulation of beta 2-Adrenergic Receptor Increases Cystic Fibrosis Transmembrane Conductance Regulator Expression in Human Airway Epithelial Cells through a cAMP/Protein Kinase A-independent Pathway
J. Biol. Chem., May 2, 2003; 278(19): 17320 - 17327.
[Abstract] [Full Text] [PDF]

Home page
 J. Biol. Chem.Home page
C. Perez-Terzic, A. M. Gacy, R. Bortolon, P. P. Dzeja, M. Puceat, M. Jaconi, F. G. Prendergast, and A. Terzic
Directed Inhibition of Nuclear Import in Cellular Hypertrophy
J. Biol. Chem., June 1, 2001; 276(23): 20566 - 20571.
[Abstract] [Full Text] [PDF]


HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS
Visit Other APS Journals Online