Results 131 to 140 of about 2,418 (178)
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A functional CFTR-NBF1 is required for ROMK2-CFTR interaction
American Journal of Physiology-Renal Physiology, 1997In a previous study on inside-out patches of Xenopus oocytes, we demonstrated that the cystic fibrosis transmembrane conductance regulator (CFTR) enhances the glibenclamide sensitivity of a coexpressed inwardly rectifying K+ channel, ROMK2 (C. M. McNicholas, W. B. Guggino, E. M. Schwiebert, S. C. Hebert, G. Giebisch, and M. E. Egan. Proc. Natl.
C M, McNicholas +6 more
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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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Pfl�gers Archiv European Journal of Physiology, 2001
Cystic fibrosis (CF) is a complex disease affecting epithelial ion transport. There are not many diseases like CF that have triggered such intense research activities. The complexity of the disease is due to mutations in the CFTR protein, now known to be a Cl(-) channel and a regulator of other transport proteins. The various interactions and the large
Greger, Rainer F +8 more
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Cystic fibrosis (CF) is a complex disease affecting epithelial ion transport. There are not many diseases like CF that have triggered such intense research activities. The complexity of the disease is due to mutations in the CFTR protein, now known to be a Cl(-) channel and a regulator of other transport proteins. The various interactions and the large
Greger, Rainer F +8 more
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Glutathione permeability of CFTR
American Journal of Physiology-Cell Physiology, 1998The cystic fibrosis transmembrane conductance regulator (CFTR) forms an ion channel that is permeable both to Cl− and to larger organic anions. Here we show, using macroscopic current recording from excised membrane patches, that the anionic antioxidant tripeptide glutathione is permeant in the CFTR channel.
P, Linsdell, J W, Hanrahan
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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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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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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.
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