Results 271 to 280 of about 81,604 (317)

Glutathione permeability of CFTR

American Journal of Physiology-Cell Physiology, 1998
The 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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Cystic fibrosis and CFTR

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, Schreiber, Rainer, Mall, Marcus, Wissner, Andreas, Hopf, Anna, Briel, Matthias, Bleich, Markus, Warth, Richard, Kunzelmann, Karl   +8 more
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Diagnostic CFTR mutation analysis

Expert Opinion on Medical Diagnostics, 2008
The aim of this review is to provide an overview of the current scientific thinking on the molecular diagnosis of cystic fibrosis (CF). The paper describes current practices and technologies regarding the molecular analysis of the CF disease-causing mutations.
D’Apice, MR, NOVELLI, GIUSEPPE, SANGIUOLO, FEDERICA CARLA   +2 more
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PIKfyve upregulates CFTR activity

Biochemical and Biophysical Research Communications, 2009
The cystic fibrosis transmembrane conductance regulator (CFTR) is a cAMP-activated Cl(-) channel critically important in Cl(-) secreting epithelia. Mutations in the CFTR gene, such as (DeltaF508)CFTR leads to cystic fibrosis, a severe disease with defective Cl(-) secretion. CFTR is stimulated by the serum and glucocorticoid-inducible kinase SGK1.
Gehring, E-M, Lam, RS, Siraskar, G, Koutsouki, E, Seebohm, G, Ureche, ON, Ureche, L, Baltaev, R, Tavaré, JM, Lang, F   +9 more
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Mechanosensitive gating of CFTR

Nature Cell Biology, 2010
Cystic 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, Wang, Dong, Duan, Yuanyuan, Loy, Michael M.T., Chan, Hsiao Chang, Huang, Pingbo   +5 more
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Quantification of CFTR Transcripts

2011
Quantification 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, Luka A, Clarke, Margarida D, Amaral   +2 more
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CFTR: Interacting With Everything?

Physiology, 2001
More 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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Expanding CFTR Modulator Testing to Carriers of CFTR Variants

Annals of the American Thoracic Society, 2021
Preston E, Bratcher, Pamela L, Zeitlin
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Ten Years With CFTR

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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CFTR Regulation by Phosphorylation

2011
The 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, J Darwin, King, Kenneth R, Hallows   +2 more
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