Role of Cftr genotype in the response to chronic Pseudomonas aeruginosa lung infection in mice

AM van Heeckeren, MD Schluchter… - … of Physiology-Lung …, 2004 - journals.physiology.org
Patients with cystic fibrosis have a lesion in the cystic fibrosis transmembrane conductance
regulator gene (CFTR), which is associated with abnormal regulation of other ion channels …

Calcium-stimulated Clsecretion in Calu-3 human airway cells requires CFTR

S Moon, M Singh, ME Krouse… - American Journal of …, 1997 - journals.physiology.org
Human airway serous cells secrete antibiotic-rich fluid, but, in cystic fibrosis (CF), Cl−-
dependent fluid secretion is impaired by defects in CF transmembrane conductance …

Treatment of cystic fibrosis airway cells with CFTR modulators reverses aberrant mucus properties via hydration

CB Morrison, KM Shaffer, KC Araba… - European …, 2022 - Eur Respiratory Soc
Question Cystic fibrosis (CF) is characterised by the accumulation of viscous adherent
mucus in the lungs. While several hypotheses invoke a direct relationship with cystic fibrosis …

[HTML][HTML] CFTR delivery to 25% of surface epithelial cells restores normal rates of mucus transport to human cystic fibrosis airway epithelium

L Zhang, B Button, SE Gabriel, S Burkett, Y Yan… - PLoS …, 2009 - journals.plos.org
Dysfunction of CFTR in cystic fibrosis (CF) airway epithelium perturbs the normal regulation
of ion transport, leading to a reduced volume of airway surface liquid (ASL), mucus …

CFTR with a partially deleted R domain corrects the cystic fibrosis chloride transport defect in human airway epithelia in vitro and in mouse nasal mucosa in vivo

LS Ostedgaard, J Zabner… - Proceedings of the …, 2002 - National Acad Sciences
In developing gene therapy for cystic fibrosis (CF) airways disease, a transgene encoding a
partially deleted CF transmembrane conductance regulator (CFTR) Cl− channel could be of …

[HTML][HTML] Cigarette smoke exposure induces CFTR internalization and insolubility, leading to airway surface liquid dehydration

LA Clunes, CM Davies, RD Coakley… - The FASEB …, 2012 - ncbi.nlm.nih.gov
Cigarette smoke (CS) exposure induces mucus obstruction and the development of chronic
bronchitis (CB). While many of these responses are determined genetically, little is known …

[HTML][HTML] The Cystic Fibrosis Transmembrane Conductance Regulator Impedes Proteolytic Stimulation of the Epithelial Na+ Channel*♦

M Gentzsch, H Dang, Y Dang… - Journal of Biological …, 2010 - ASBMB
Cystic fibrosis (CF) is caused by mutations in the CF transmembrane conductance regulator
(CFTR) that prevent its proper folding and trafficking to the apical membrane of epithelial …

Cystic fibrosis: emergence of highly effective targeted therapeutics and potential clinical implications

MA Mall, N Mayer-Hamblett… - American journal of …, 2020 - atsjournals.org
Cystic fibrosis (CF) remains the most common life-shortening hereditary disease in white
populations, with high morbidity and mortality related to chronic airway mucus obstruction …

Lack of Cystic Fibrosis Transmembrane Conductance Regulator in CD3+ Lymphocytes Leads to Aberrant Cytokine Secretion and Hyperinflammatory Adaptive …

C Mueller, SA Braag, A Keeler, C Hodges… - American journal of …, 2011 - atsjournals.org
Cystic fibrosis (CF), the most common fatal monogenic disease in the United States, results
from mutations in CF transmembrane conductance regulator (CFTR), a chloride channel …

[HTML][HTML] Basal nucleotide levels, release, and metabolism in normal and cystic fibrosis airways

SH Donaldson, ER Lazarowski, M Picher… - Molecular …, 2000 - Springer
Background Cystic fibrosis (CF) is a syndrome caused by mutations in the cystic fibrosis
transmembrane regulator (CFTR) gene. Despite advances in our understanding of the …