Maxi-K (BK) Channels Contribute to Nucleotide-Stimulated Chloride Secretion and Mucociliary Transport in Normal and Cystic Fibrosis Airway Cells.

2009 ◽  
Author(s):  
D Manzanares ◽  
M Valencia Gattas ◽  
P Ivonnet ◽  
GE Conner ◽  
M Salathe
Keyword(s):  
Cystic Fibrosis ◽  
Chloride Secretion ◽  
Bk Channels ◽  
Mucociliary Transport ◽  
Cystic Fibrosis Airway ◽  
Airway Cells

scholarly journals Trimethylangelicin promotes the functional rescue of mutant F508del CFTR protein in cystic fibrosis airway cells

2014 ◽  
Vol 307 (1) ◽  
pp. L48-L61 ◽  
Author(s):  
Maria Favia ◽  
Maria T. Mancini ◽  
Valentino Bezzerri ◽  
Lorenzo Guerra ◽  
Onofrio Laselva ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Cell Surface ◽  
Surface Expression ◽  
Chloride Secretion ◽  
Cell Surface Expression ◽  
Chloride Permeability ◽  
Cell Monolayers ◽  
Cystic Fibrosis Airway ◽  
Cftr Protein ◽  
Airway Cells

Cystic fibrosis transmembrane conductance regulator (CFTR) carrying the F508del mutation is retained in endoplasmic reticulum and fails to traffic to the cell surface where it functions as a protein kinase A (PKA)-activated chloride channel. Pharmacological correctors that rescue the trafficking of F508del CFTR may overcome this defect; however, the rescued F508del CFTR still displays reduced chloride permeability. Therefore, a combined administration of correctors and potentiators of the gating defect is ideal. We recently found that 4,6,4′-trimethylangelicin (TMA), besides inhibiting the expression of the IL-8 gene in airway cells in which the inflammatory response was challenged with Pseudomonas aeruginosa, also potentiates the cAMP/PKA-dependent activation of wild-type CFTR or F508del CFTR that has been restored to the plasma membrane. Here, we demonstrate that long preincubation with nanomolar concentrations of TMA is able to effectively rescue both F508del CFTR-dependent chloride secretion and F508del CFTR cell surface expression in both primary or secondary airway cell monolayers homozygous for F508del mutation. The correction effect of TMA seems to be selective for CFTR and persisted for 24 h after washout. Altogether, the results suggest that TMA, besides its anti-inflammatory and potentiator activities, also displays corrector properties.

scholarly journals Respiratory Syncytial Virus Infection Disrupts Monolayer Integrity and Function in Cystic Fibrosis Airway Cells

Viruses ◽  
2013 ◽  
Vol 5 (9) ◽  
pp. 2260-2271 ◽  
Author(s):  
Michele Kong ◽  
Patrick Maeng ◽  
Jeong Hong ◽  
Rhonda Szczesniak ◽  
Eric Sorscher ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Respiratory Syncytial Virus ◽  
Virus Infection ◽  
Respiratory Syncytial Virus Infection ◽  
Cystic Fibrosis Airway ◽  
Syncytial Virus ◽  
And Function ◽  
Airway Cells

Effects of Azithromycin on Glutathione S-Transferases in Cystic Fibrosis Airway Cells

2009 ◽  
Vol 41 (2) ◽  
pp. 199-206 ◽  
Author(s):  
Gabriella Bergamini ◽  
Cristina Cigana ◽  
Claudio Sorio ◽  
Marco Della Peruta ◽  
Alfonso Pompella ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Cystic Fibrosis Airway ◽  
Glutathione S Transferases ◽  
Airway Cells

scholarly journals Extracellular Zinc and ATP Restore Chloride Secretion across Cystic Fibrosis Airway Epithelia by Triggering Calcium Entry

2003 ◽  
Vol 279 (11) ◽  
pp. 10720-10729 ◽  
Author(s):  
Ákos Zsembery ◽  
James A. Fortenberry ◽  
Lihua Liang ◽  
Zsuzsa Bebok ◽  
Torry A. Tucker ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Chloride Secretion ◽  
Calcium Entry ◽  
Airway Epithelia ◽  
Cystic Fibrosis Airway

Phosphorylation fails to activate chloride channels from cystic fibrosis airway cells

Nature ◽  
1987 ◽  
Vol 330 (6150) ◽  
pp. 752-754 ◽  
Author(s):  
Robert A. Schoumacher ◽  
Richard L. Shoemaker ◽  
Dan R. Halm ◽  
E. Ann Tallant ◽  
Robert W. Wallace ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Chloride Channels ◽  
Cystic Fibrosis Airway ◽  
Airway Cells

scholarly journals Analysis of ClC-2 channels as an alternative pathway for chloride conduction in cystic fibrosis airway cells

1998 ◽  
Vol 95 (7) ◽  
pp. 3879-3884 ◽  
Author(s):  
E. M. Schwiebert ◽  
L. P. Cid-Soto ◽  
D. Stafford ◽  
M. Carter ◽  
C. J. Blaisdell ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Alternative Pathway ◽  
Cystic Fibrosis Airway ◽  
Airway Cells
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