Abnormal surface liquid pH regulation by cultured cystic fibrosis bronchial epithelium

RD Coakley, BR Grubb, AM Paradiso… - Proceedings of the …, 2003 - National Acad Sciences
Cystic fibrosis (CF) transmembrane conductance regulator (CFTR)-dependent airway
epithelial bicarbonate transport is hypothesized to participate in airway surface liquid pH …

Chloride secretory response of cystic fibrosis human airway epithelia. Preservation of calcium but not protein kinase C-and A-dependent mechanisms.

RC Boucher, EH Cheng, AM Paradiso… - The Journal of …, 1989 - Am Soc Clin Investig
Because the defect in Cl-secretion exhibited by cystic fibrosis (CF) epithelia reflects
regulatory rather than conductive abnormalities of an apical membrane Cl-channel, we …

[HTML][HTML] Loss of CFTR chloride channels alters salt absorption by cystic fibrosis airway epithelia in vitro

J Zabner, JJ Smith, PH Karp, JH Widdicombe… - Molecular cell, 1998 - cell.com
Cystic fibrosis (CF) is caused by the loss of functional CFTR Cl− channels. However, it is not
understood how this defect disrupts salt and liquid movement in the airway or whether it …

[HTML][HTML] Airway surface liquid acidification initiates host defense abnormalities in Cystic Fibrosis

J Simonin, E Bille, G Crambert, S Noel, E Dreano… - Scientific reports, 2019 - nature.com
Cystic fibrosis (CF) is caused by defective Cystic Fibrosis Transmembrane Conductance
Regulator (CFTR) protein. Morbidity is mainly due to early airway infection. We hypothesized …

Regulation of Murine Airway Surface Liquid Volume by CFTR and Ca2+-activated Cl Conductances

R Tarran, ME Loewen, AM Paradiso, JC Olsen… - The Journal of general …, 2002 - rupress.org
Two Cl− conductances have been described in the apical membrane of both human and
murine proximal airway epithelia that are thought to play predominant roles in airway …

[HTML][HTML] Evidence for periciliary liquid layer depletion, not abnormal ion composition, in the pathogenesis of cystic fibrosis airways disease

H Matsui, BR Grubb, R Tarran, SH Randell, JT Gatzy… - Cell, 1998 - cell.com
The pathogenesis of cystic fibrosis (CF) airways infection is unknown. Two hypotheses,"
hypotonic [low salt]/defensin" and" isotonic volume transport/mucus clearance," attempt to …

Hyperacidity of secreted fluid from submucosal glands in early cystic fibrosis

Y Song, D Salinas, DW Nielson… - American Journal of …, 2006 - journals.physiology.org
Prior studies have shown that fluid secretions from airway submucosal glands in cystic
fibrosis (CF) are reduced and hyperviscous, possibly contributing to the pathogenesis of CF …

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

AS Verkman, Y Song… - American Journal of …, 2003 - journals.physiology.org
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 …

Multiple modes of regulation of airway epithelial chloride secretion by extracellular ATP

MJ Stutts, JG Fitz, AM Paradiso… - American Journal of …, 1994 - journals.physiology.org
Cultured normal and cystic fibrosis (CF) airway epithelia were exposed to 5'-(N-
ethylcarboxamido)-adenosine (NECA), ATP, or ionomycin. NECA activated a sustained, 4, 4' …

CFTR and lung homeostasis

JF Collawn, S Matalon - American Journal of Physiology …, 2014 - journals.physiology.org
CFTR is a cAMP-activated chloride and bicarbonate channel that is critical for lung
homeostasis. Decreases in CFTR expression have dire consequences in cystic fibrosis (CF) …