Cystic Fibrosis Transmembrane Conductance Regulator

Known as: CFTR, CFTR Protein, cystic fibrosis transmembrane conductance regulator (CFTR) protein 
A chloride channel that regulates secretion in many exocrine tissues. Abnormalities in the CFTR gene have been shown to cause cystic fibrosis. (Hum… (More)
National Institutes of Health

Related topics

Related topics

12 relations
CFTR geneCystic FibrosisIn BloodProcess of secretion
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Broader (1)

Chloride Channels

Narrower (1)

cystic fibrosis transmembrane conductance regulator delta F508

Papers overview

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Highly Cited
2014
Highly Cited
2014
Clinical mechanism of the cystic fibrosis transmembrane conductance regulator potentiator ivacaftor in G551D-mediated cystic fibrosis.
RATIONALE Ivacaftor is a cystic fibrosis transmembrane conductance regulator (CFTR) potentiator recently approved for patients… (More)
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Highly Cited
2010
Highly Cited
2010
Ataluren (PTC124) induces cystic fibrosis transmembrane conductance regulator protein expression and activity in children with nonsense mutation cystic fibrosis.
RATIONALE Nonsense (premature stop codon) mutations in mRNA for the cystic fibrosis transmembrane conductance regulator (CFTR… (More)
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Highly Cited
2004
Highly Cited
2004
Structure of nucleotide-binding domain 1 of the cystic fibrosis transmembrane conductance regulator.
Cystic fibrosis transmembrane conductance regulator (CFTR) is an ATP-binding cassette (ABC) transporter that functions as a… (More)
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Highly Cited
2002
Highly Cited
2002
Five percent of normal cystic fibrosis transmembrane conductance regulator mRNA ameliorates the severity of pulmonary disease in cystic fibrosis.
Estimates of the level of transcripts from the cystic fibrosis (CF) transmembrane conductance regulator (CFTR) gene required to… (More)
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Highly Cited
1996
Highly Cited
1996
Modulation of disease severity in cystic fibrosis transmembrane conductance regulator deficient mice by a secondary genetic factor
Mice that have been made deficient for the cystic fibrosis transmembrane conductance regulator (Cftr) usually die of intestinal… (More)
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Highly Cited
1994
Highly Cited
1994
Bicarbonate conductance and pH regulatory capability of cystic fibrosis transmembrane conductance regulator.
The cystic fibrosis transmembrane conductance regulator (CFTR) is an epithelial Cl- channel regulated by protein kinase A. The… (More)
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Highly Cited
1992
Highly Cited
1992
In vivo transfer of the human cystic fibrosis transmembrane conductance regulator gene to the airway epithelium
Direct transfer of the normal cystic fibrosis (CF) transmembrane conductance regulator (CFTR) gene to airway epithelium was… (More)
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Highly Cited
1992
Highly Cited
1992
Localization of cystic fibrosis transmembrane conductance regulator in chloride secretory epithelia.
Cystic fibrosis is caused by mutations in the gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR). To… (More)
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Highly Cited
1991
Highly Cited
1991
Localization of the cystic fibrosis transmembrane conductance regulator in pancreas.
Cystic fibrosis (CF) is characterized by an abnormality in cAMP-regulated chloride transport that results from a primary defect… (More)
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Highly Cited
1990
Highly Cited
1990
Expression of cystic fibrosis transmembrane conductance regulator corrects defective chloride channel regulation in cystic fibrosis airway epithelial cells
The cystic fibrosis transmembrane conductance regulator (CFTR) was expressed in cultured cystic fibrosis airway epithelial cells… (More)
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