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 …
regulator gene (CFTR), which is associated with abnormal regulation of other ion channels …
Calcium-stimulated Cl−secretion 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 …
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
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 …
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 …
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 …
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 …
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 …
(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 …
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 …
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 …
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 …
transmembrane regulator (CFTR) gene. Despite advances in our understanding of the …