Reduced GM1 ganglioside in CFTR-deficient human airway cells results in decreased β1-integrin signaling and delayed wound repair

Y Itokazu, RE Pagano, AS Schroeder… - … of Physiology-Cell …, 2014 - journals.physiology.org
Loss of cystic fibrosis transmembrane conductance regulator (CFTR) function reduces
chloride secretion and increases sodium uptake, but it is not clear why CFTR mutation also …

Cystic fibrosis transmembrane conductance regulator is involved in airway epithelial wound repair

KR Schiller, PJ Maniak… - American Journal of …, 2010 - journals.physiology.org
The role of cystic fibrosis (CF) transmembrane conductance regulator (CFTR) in airway
epithelial wound repair was investigated using normal human bronchial epithelial (NHBE) …

Pathophysiology of lung disease and wound repair in cystic fibrosis

M Conese, S Di Gioia - Pathophysiology, 2021 - mdpi.com
Cystic fibrosis (CF) is an autosomal recessive, life-threatening condition affecting many
organs and tissues, the lung disease being the chief cause of morbidity and mortality …

Improvement of defective cystic fibrosis airway epithelial wound repair after CFTR rescue

NTN Trinh, O Bardou, A Privé, E Maillé… - European …, 2012 - Eur Respiratory Soc
Airway damage and remodelling are important components of lung pathology progression in
cystic fibrosis (CF). Although repair mechanisms are engaged to restore the epithelial …

CFTR inhibition mimics the cystic fibrosis inflammatory profile

A Perez, AC Issler, CU Cotton… - … of Physiology-Lung …, 2007 - journals.physiology.org
Primary airway epithelial cells grown in air-liquid interface differentiate into cultures that
resemble native epithelium morphologically, express ion transport similar to those in vivo …

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 …

Tgf-beta downregulation of distinct chloride channels in cystic fibrosis-affected epithelia

H Sun, WT Harris, S Kortyka, K Kotha, AJ Ostmann… - PloS one, 2014 - journals.plos.org
Rationale The cystic fibrosis transmembrane conductance regulator (CFTR) and Calcium-
activated Chloride Conductance (CaCC) each play critical roles in maintaining normal …

[HTML][HTML] Deleterious impact of hyperglycemia on cystic fibrosis airway ion transport and epithelial repair

C Bilodeau, O Bardou, É Maillé, Y Berthiaume… - Journal of Cystic …, 2016 - Elsevier
Background Cystic fibrosis (CF)-related diabetes (CFRD) is associated with faster
pulmonary function decline. Thus, we evaluated the impact of hyperglycemia on airway …

[HTML][HTML] Purinergic signaling underlies CFTR control of human airway epithelial cell volume

GM Braunstein, A Zsembery, TA Tucker… - Journal of Cystic …, 2004 - Elsevier
Background: Loss of cystic fibrosis transmembrane conductance regulator (CFTR) function
in cystic fibrosis (CF) causes dysregulation of multiple ion channels, water channels, and …

Sphingomyelinase decreases transepithelial anion secretion in airway epithelial cells in part by inhibiting CFTR‐mediated apical conductance

KA Cottrill, RJ Peterson, CF Lewallen… - Physiological …, 2021 - Wiley Online Library
The cystic fibrosis transmembrane conductance regulator (CFTR) is an anion channel
whose dysfunction causes cystic fibrosis (CF). The loss of CFTR function in pulmonary …