The cystic fibrosis transmembrane conductance regulator gene

LC Tsui - American journal of respiratory and critical care …, 1995 - atsjournals.org
… Since identification of the gene responsible for cystic fibrosis (CF) in 1989, significant … the
CF gene, referred to as the CF transmembrane conductance regulator (CFTR), indicate that the …

Expression and characterization of the cystic fibrosis transmembrane conductance regulator

RJ Gregory, SH Cheng, DP Rich, J Marshall, S Paul… - Nature, 1990 - nature.com
CYSTIC fibrosis (CF) is a common lethal genetic disease that manifests itself in airway and
other epithelial cells as defective chloride ion absorption and secretion 1,2 , resulting at least …

[HTML][HTML] The cystic fibrosis transmembrane conductance regulator: an intriguing protein with pleiotropic functions

A Vankeerberghen, H Cuppens, JJ Cassiman - Journal of Cystic Fibrosis, 2002 - Elsevier
Cystic fibrosis is a frequent autosomal recessive disorder that is caused by the … , the cystic
fibrosis transmembrane conductance regulator. The protein is found in the apical membrane of …

[HTML][HTML] Atomic structure of the cystic fibrosis transmembrane conductance regulator

Z Zhang, J Chen - Cell, 2016 - cell.com
The cystic fibrosis transmembrane conductance regulator (CFTR) is an anion channel evolved
from the ATP-binding cassette (ABC) transporter family. In this study, we determined the …

Targeting a genetic defect: cystic fibrosis transmembrane conductance regulator modulators in cystic fibrosis

N Derichs - European Respiratory Review, 2013 - Eur Respiratory Soc
Cystic fibrosis (CF) is caused by genetic mutations that affect the cystic fibrosis transmembrane
conductance regulator (… membrane of epithelial cells, as well as influencing the gating or …

Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature-sensitive

GM Denning, MP Anderson, JF Amara, J Marshall… - nature, 1992 - nature.com
CYSTIC fibrosis transmembrane conductance regulator (CFTR) is a plasma membrane CIT
channel … Mutations in CFTR cause cystic fibrosis"" partly through loss of cAMPregulated ClT …

[HTML][HTML] Phosphorylation of the cystic fibrosis transmembrane conductance regulator.

MR Picciotto, JA Cohn, G Bertuzzi, P Greengard… - Journal of Biological …, 1992 - Elsevier
… be responsible for regulation of CFTR … cystic fibrosis; CFTR, cystic tion by PKA and PKC in
CF-2 and find that within CF-2, consensus sites fibrosis transmembrane conductance regulator

Bicarbonate conductance and pH regulatory capability of cystic fibrosis transmembrane conductance regulator.

JH Poulsen, H Fischer, B Illek… - Proceedings of the …, 1994 - National Acad Sciences
… The cystic fibrosis transmembrane conductance regulator (CFTR) is an epithelial Cl- channel
regulated by cAMPdependent protein kinaseA (1-3). The most common mutation in CF is …

Identification and regulation of the cystic fibrosis transmembrane conductance regulator-generated chloride channel.

HA Berger, MP Anderson, RJ Gregory… - The Journal of …, 1991 - Am Soc Clin Investig
… open apical membrane CL- channels (1, 2). Expression ofwildtype CF transmembrane
conductance regulator (CFTR) (3) in CF epithelia corrects the defect in cAMP-regulated Cl- perme…

Expression of cystic fibrosis transmembrane conductance regulator corrects defective chloride channel regulation in cystic fibrosis airway epithelial cells

DP Rich, MP Anderson, RJ Gregory, SH Cheng, S Paul… - Nature, 1990 - nature.com
… The cystic fibrosis transmembrane conductance regulator (CFTR) was expressed in cultured
cystic fibrosis airway epithelial cells and CIT channel activation assessed in single cells …