Chloride Channels in Cystic Fibrosis

1994 ◽  
pp. 500-525
Author(s):  
Carole M. Liedtke
Keyword(s):  
Cystic Fibrosis ◽  
Chloride Channels

Chloride channels in normal and cystic fibrosis human erythrocyte membrane

2007 ◽  
Vol 39 (1) ◽  
pp. 24-34 ◽  
Author(s):  
Gaëtan Decherf ◽  
Guillaume Bouyer ◽  
Stéphane Egée ◽  
Serge L.Y. Thomas
Keyword(s):  
Cystic Fibrosis ◽  
Erythrocyte Membrane ◽  
Human Erythrocyte ◽  
Chloride Channels ◽  
Human Erythrocyte Membrane

ClC-5: ontogeny of an alternative chloride channel in respiratory epithelia

2002 ◽  
Vol 282 (3) ◽  
pp. L501-L507 ◽  
Author(s):  
Rebecca D. Edmonds ◽  
Ian V. Silva ◽  
William B. Guggino ◽  
Robert B. Butler ◽  
Pamela L. Zeitlin ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Lung Disease ◽  
Chloride Channel ◽  
Chloride Channels ◽  
Chloride Transport ◽  
Premature Death ◽  
Fetal Lung ◽  
Chloride Secretion ◽  
Normal Lung ◽  
Ribonuclease Protection Assay

Chloride transport is critical to many functions of the lung. Molecular defects in the best-known chloride channel, cystic fibrosis transmembrane conductance regulator (CFTR), lead to impaired function of airway defensins, hydration of airway surface fluid, and mucociliary clearance leading to chronic lung disease, and premature death, but do not cause defects in lung development. We examined the expression of one member of the ClC family of volume- and voltage-regulated channels using the ribonuclease protection assay and Western blot analysis in rats. ClC-5 mRNA and protein are most strongly expressed in the fetal lung, and expression is maintained although downregulated postnatally. In addition, using immunocytochemistry, we find that ClC-5 is predominantly expressed along the luminal surface of the airway epithelium, suggesting that ClC-5 may participate in lung chloride secretion. Identifying candidate genes for critical ion transport functions is essential for understanding normal lung morphogenesis and the pathophysiology of several lung diseases. In addition, the manipulation of non-CFTR chloride channels may provide a viable approach for treating cystic fibrosis lung disease.

Chloride channels, Golgi pH and cystic fibrosis

1992 ◽  
Vol 2 (2) ◽  
pp. 35-37 ◽  
Author(s):  
Jonathan Barasch ◽  
Qais Al-Awqati
Keyword(s):  
Cystic Fibrosis ◽  
Chloride Channels

scholarly journals State-dependent Inhibition of Cystic Fibrosis Transmembrane Conductance Regulator Chloride Channels by a Novel Peptide Toxin

2007 ◽  
Vol 282 (52) ◽  
pp. 37545-37555 ◽  
Author(s):  
Matthew D. Fuller ◽  
Christopher H. Thompson ◽  
Zhi-Ren Zhang ◽  
Cody S. Freeman ◽  
Eszter Schay ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Chloride Channels ◽  
Transmembrane Conductance Regulator ◽  
Transmembrane Conductance ◽  
State Dependent ◽  
Dependent Inhibition ◽  
Peptide Toxin ◽  
Cystic Fibrosis Transmembrane

scholarly journals Defective organellar acidification as a cause of cystic fibrosis lung disease: reexamination of a recurring hypothesis

2009 ◽  
Vol 296 (6) ◽  
pp. L859-L867 ◽  
Author(s):  
Peter M. Haggie ◽  
A. S. Verkman
Keyword(s):  
Cystic Fibrosis ◽  
Epithelial Cells ◽  
Lung Disease ◽  
Alveolar Macrophages ◽  
Chloride Channels ◽  
Loss Of Function ◽  
Cellular Mechanisms ◽  
Sodium Efflux ◽  
Cystic Fibrosis Lung Disease ◽  
Respiratory Epithelial

The cellular mechanisms by which loss-of-function mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) chloride channel produce cystic fibrosis (CF) lung disease remain uncertain. Defective organellar function has been proposed as an important determinant in the pathogenesis of CF lung disease. According to one hypothesis, reduced CFTR chloride conductance in organelles in CF impairs their acidification by preventing chloride entry into the organelle lumen, which is needed to balance the positive charge produced by proton entry. According to a different hypothesis, CFTR mutation hyperacidifies organelles by an indirect mechanism involving unregulated sodium efflux through epithelial sodium channels. There are reports of defective Golgi, endosomal and lysosomal acidification in CF epithelial cells, defective phagolysosomal acidification in CF alveolar macrophages, and organellar hyperacidification in CF respiratory epithelial cells. The common theme relating too high or low organellar pH to cellular dysfunction and CF pathogenesis is impaired functioning of organellar enzymes, such as those involved in ceramide metabolism and protein processing in epithelial cells and antimicrobial activity in alveolar macrophages. We review here the evidence for defective organellar acidification in CF. Significant technical and conceptual concerns are discussed regarding the validity of data showing too high/low organellar pH in CF cells, and rigorous measurements of organellar pH in CF cells are reviewed that fail to support defective organellar acidification in CF. Indeed, there is an expanding body of evidence supporting the involvement of non-CFTR chloride channels in organellar acidification. We conclude that biologically significant involvement of CFTR in organellar acidification is unlikely.

Sign in / Sign up

Export Citation Format

Share Document