Airway acidification initiates host defense abnormalities in cystic fibrosis mice
Cystic fibrosis (CF) is caused by mutations in the gene that encodes the cystic fibrosis
transmembrane conductance regulator (CFTR) anion channel. In humans and pigs, the loss …
transmembrane conductance regulator (CFTR) anion channel. In humans and pigs, the loss …
Small-molecule ion channels increase host defences in cystic fibrosis airway epithelia
KA Muraglia, RS Chorghade, BR Kim, XX Tang… - Nature, 2019 - nature.com
Loss-of-function mutations in the cystic fibrosis transmembrane conductance regulator
(CFTR) compromise epithelial HCO3− and Cl− secretion, reduce airway surface liquid pH …
(CFTR) compromise epithelial HCO3− and Cl− secretion, reduce airway surface liquid pH …
Reduced airway surface pH impairs bacterial killing in the porcine cystic fibrosis lung
AA Pezzulo, XX Tang, MJ Hoegger, MH Abou Alaiwa… - Nature, 2012 - nature.com
Cystic fibrosis (CF) is a life-shortening disease caused by mutations in the cystic fibrosis
transmembrane conductance regulator (CFTR) gene. Although bacterial lung infection and …
transmembrane conductance regulator (CFTR) gene. Although bacterial lung infection and …
Altered fluid transport across airway epithelium in cystic fibrosis
C Jiang, WE Finkbeiner, JH Widdicombe, PB McCray Jr… - Science, 1993 - science.org
In cystic fibrosis (CF), absence or dysfunction of a phosphorylation-regulated chloride
channel [CF transmembrane conductance regulator (CFTR)] leads to the loss or reduction of …
channel [CF transmembrane conductance regulator (CFTR)] leads to the loss or reduction of …
Increased airway epithelial Na+ absorption produces cystic fibrosis-like lung disease in mice
Mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene result in
defective epithelial cAMP-dependent Cl− secretion and increased airway Na+ absorption …
defective epithelial cAMP-dependent Cl− secretion and increased airway Na+ absorption …
Defective epithelial chloride transport in a gene-targeted mouse model of cystic fibrosis
LL Clarke, BR Grubb, SE Gabriel, O Smithies… - Science, 1992 - science.org
The cystic fibrosis transmembrane conductance regulator (CFTR) gene encodes an
adenosine 3′, 5′-monophosphate (cyclic AMP)—activated chloride channel. In cystic …
adenosine 3′, 5′-monophosphate (cyclic AMP)—activated chloride channel. In cystic …
Role of mutant CFTR in hypersusceptibility of cystic fibrosis patients to lung infections
GB Pier, M Grout, TS Zaidi, JC Olsen, LG Johnson… - Science, 1996 - science.org
Cystic fibrosis (CF) patients are hypersusceptible to chronic Pseudomonas aeruginosa lung
infections. Cultured human airway epithelial cells expressing the ΔF508 allele of the cystic …
infections. Cultured human airway epithelial cells expressing the ΔF508 allele of the cystic …
Failure of the cystic fibrosis transmembrane conductance regulator to conduct ATP
MM Reddy, PM Quinton, C Haws, JJ Wine… - Science, 1996 - science.org
The cystic fibrosis transmembrane conductance regulator (CFTR) is a chloride ion channel
regulated by protein kinase A and adenosine triphosphate (ATP). Loss of CFTR-mediated …
regulated by protein kinase A and adenosine triphosphate (ATP). Loss of CFTR-mediated …
An animal model for cystic fibrosis made by gene targeting
JN Snouwaert, KK Brigman, AM Latour, NN Malouf… - Science, 1992 - science.org
Cystic fibrosis results from defects in the gene encoding a cyclic adenosine monophosphate-
dependent chloride ion channel known as the cystic fibrosis transmembrane conductance …
dependent chloride ion channel known as the cystic fibrosis transmembrane conductance …
[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 …
understood how this defect disrupts salt and liquid movement in the airway or whether it …