Results 181 to 190 of about 31,972 (221)
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CFTR: Interacting With Everything?
Physiology, 2001More than 1,300 different mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) are the cause for cystic fibrosis. CFTR is in charge of proper secretion and absorption of electrolytes, and thus the disease is characterized by defective epithelial Cl–secretion and enhanced Na+absorption. Recent studies show that CFTR interacts with
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Mechanosensitive gating of CFTR
Nature Cell Biology, 2010Cystic fibrosis transmembrane conductance regulator (CFTR) is an anion and intracellular ligand-gated channel associated with cystic fibrosis, a lethal genetic disorder common among Caucasians. Here we show that CFTR is robustly activated by membrane stretch induced by negative pressures as small as 5 mmHg at the single-channel, cellular and tissue ...
Zhang, Wei Kevin +5 more
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The gating of the CFTR channel
Cellular and Molecular Life Sciences, 2016Cystic fibrosis transmembrane conductance regulator (CFTR) is an anion channel expressed in the apical membrane of epithelia. Mutations in the CFTR gene are the cause of cystsic fibrosis. CFTR is the only ABC-protein that constitutes an ion channel pore forming subunit.
OSCAR Moran, Moran OSCAR
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Direct interaction of a CFTR potentiator and a CFTR corrector with phospholipid bilayers
European Biophysics Journal, 2014Cystic fibrosis transmembrane conductance regulator (CFTR) potentiators and correctors are new drugs that target the basic CFTR protein defect and are expected to benefit cystic fibrosis patients. To optimize the substances so far proposed for human use, and to minimise unwanted side effects, it is essential to investigate possible interactions between
Baroni D +3 more
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Haplotype analysis of the CFTR gene on normal and mutant CFTR genes
Mutation Research - Fundamental and Molecular Mechanisms of Mutagenesis, 2020Mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene are responsible for Cystic Fibrosis (CF) disease. Since the distribution of polymorphisms varies among populations, a comparison between the frequency of CFTR polymorphisms in patients and healthy population may further identify their role in CF disease.
Nasibeh, Karimi +3 more
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Journal of Membrane Biology, 1999
The cystic fibrosis transmembrane conductance regulator (CFTR) is a cAMP-activated Cl channel that is defective in cystic fibrosis. This statement is found in most of the articles somehow dealing with CFTR. However, the initial characterization of this puzzling protein as a conductance regulator is probably more appropriate. As we have learned over the
K, Kunzelmann, R, Schreiber
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The cystic fibrosis transmembrane conductance regulator (CFTR) is a cAMP-activated Cl channel that is defective in cystic fibrosis. This statement is found in most of the articles somehow dealing with CFTR. However, the initial characterization of this puzzling protein as a conductance regulator is probably more appropriate. As we have learned over the
K, Kunzelmann, R, Schreiber
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Quantification of CFTR Transcripts
2011Quantification and analysis of CFTR transcripts is of crucial importance not only for cystic fibrosis (CF) diagnosis and prognosis, but also in evaluating the efficiency of various therapeutic approaches to CF, including gene therapy. Reverse transcription (RT) followed by quantitative polymerase chain reaction (qPCR) is at present the most sensitive ...
Anabela S, Ramalho +2 more
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Physiological Reviews, 1999
This is the second single-topic supplement to Physiological Reviews . In introducing the first supplement, Daniel Gardner indicated that the articles reported on the progress of a revolution, first recognized by the series of papers on membrane currents in nerve published by Hodgkin, Huxley ...
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This is the second single-topic supplement to Physiological Reviews . In introducing the first supplement, Daniel Gardner indicated that the articles reported on the progress of a revolution, first recognized by the series of papers on membrane currents in nerve published by Hodgkin, Huxley ...
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CFTR Regulation by Phosphorylation
2011The cystic fibrosis transmembrane conductance regulator (CFTR) is the gene product mutated in cystic fibrosis, a common lethal genetic disease characterized by abnormal electrolyte transport across epithelia. CFTR functions as an ATP-gated, phosphorylation-regulated Cl- channel that mediates agonist-stimulated apical membrane epithelial Cl- and ...
Rodrigo, Alzamora +2 more
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Annual Review of Physiology, 2000
▪ Abstract In contrast to the airways, the defects in colonic function in cystic fibrosis (CF) patients are closely related to the defect in CFTR. The gastrointestinal phenotype of CF transgenic mice closely resembles the phenotype in CF patients, which clearly indicates the crucial role of CFTR in colonic Cl− secretion and the absence of an ...
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▪ Abstract In contrast to the airways, the defects in colonic function in cystic fibrosis (CF) patients are closely related to the defect in CFTR. The gastrointestinal phenotype of CF transgenic mice closely resembles the phenotype in CF patients, which clearly indicates the crucial role of CFTR in colonic Cl− secretion and the absence of an ...
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