Cytosolic pH regulatesG Cl through control of phosphorylation states of CFTR

MM Reddy, RR Kopito… - American Journal of …, 1998 - journals.physiology.org
Our objective in this study was to determine the effect of changes in luminal and cytoplasmic
pH on cystic fibrosis transmembrane regulator (CFTR) Cl− conductance (G Cl). We …

The relationship of chronic mucin secretion to airway disease in normal and CFTR-deficient mice

VL Cressman, EM Hicks, WK Funkhouser… - American journal of …, 1998 - atsjournals.org
In the cystic fibrosis (CF) patient, lung function decreases throughout life as a result of
continuous cycles of infection, particularly with Pseudomonas aeruginosa and …

[HTML][HTML] Evidence of early increased sialylation of airway mucins and defective mucociliary clearance in CFTR-deficient piglets

I Caballero, B Ringot-Destrez, M Si-Tahar… - Journal of Cystic …, 2021 - Elsevier
Background Bacterial colonization in cystic fibrosis (CF) lungs has been directly associated
to the loss of CFTR function, and/or secondarily linked to repetitive cycles of chronic …

Overview of CF lung pathophysiology

G Cabrini, A Rimessi, M Borgatti, P Pinton… - Current Opinion in …, 2022 - Elsevier
Defects of the cystic fibrosis (CF) transmembrane conductance regulator (CFTR) protein
affect the homeostasis of chloride, bicarbonate, sodium, and water in the airway surface …

[HTML][HTML] Cystic fibrosis transmembrane conductance regulator regulates luminal Cl−/HCO3− exchange in mouse submandibular and pancreatic ducts

MG Lee, JY Choi, X Luo, E Strickland… - Journal of Biological …, 1999 - ASBMB
We have demonstrated previously the regulation of Cl−/HCO 3− exchange activity by the
cystic fibrosis transmembrane conductance regulator (CFTR) in model systems of cells …

[HTML][HTML] CGRP induction in cystic fibrosis airways alters the submucosal gland progenitor cell niche in mice

W Xie, JT Fisher, TJ Lynch, M Luo… - The Journal of …, 2011 - Am Soc Clin Investig
In cystic fibrosis (CF), a lack of functional CF transmembrane conductance regulator (CFTR)
chloride channels causes defective secretion by submucosal glands (SMGs), leading to …

[HTML][HTML] Cystic fibrosis airway secretions exhibit mucin hyperconcentration and increased osmotic pressure

AG Henderson, C Ehre, B Button… - The Journal of …, 2014 - Am Soc Clin Investig
The pathogenesis of mucoinfective lung disease in cystic fibrosis (CF) patients likely
involves poor mucus clearance. A recent model of mucus clearance predicts that mucus flow …

Dysfunctional cystic fibrosis transmembrane conductance regulator inhibits phagocytosis of apoptotic cells with proinflammatory consequences

RW Vandivier, TR Richens… - … of Physiology-Lung …, 2009 - journals.physiology.org
Cystic fibrosis (CF) is caused by mutated CF transmembrane conductance regulator (CFTR)
and is characterized by robust airway inflammation and accumulation of apoptotic cells …

[HTML][HTML] A developmental role of the cystic fibrosis transmembrane conductance regulator in cystic fibrosis lung disease pathogenesis

EN Huang, H Quach, JA Lee, J Dierolf… - Frontiers in Cell and …, 2021 - frontiersin.org
The cystic fibrosis (CF) transmembrane conductance regulator (CFTR) protein is a cAMP-
activated anion channel that is critical for regulating fluid and ion transport across the …

Differential regulation of cystic fibrosis transmembrane conductance regulator by interferon γ in mast cells and epithelial cells

M Kulka, R Dery, D Nahirney, M Duszyk… - Journal of Pharmacology …, 2005 - ASPET
Cystic fibrosis transmembrane conductance regulator (CFTR) is a cAMP-dependent chloride
channel in epithelial cells; recently, we identified it in mast cells. Previous work that we …