Airway acidification initiates host defense abnormalities in cystic fibrosis mice

VS Shah, DK Meyerholz, XX Tang, L Reznikov… - Science, 2016 - science.org
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 …

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 …

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 …

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 …

Increased airway epithelial Na+ absorption produces cystic fibrosis-like lung disease in mice

M Mall, BR Grubb, JR Harkema, WK O'Neal… - Nature medicine, 2004 - nature.com
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 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 …

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 …

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 …

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 …

[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 …