نتایج جستجو برای: cftr

تعداد نتایج: 5775  

Journal: :Cellular physiology and biochemistry : international journal of experimental cellular physiology, biochemistry, and pharmacology 2007
J Denry Sato M Christine Chapline Renee Thibodeau Raymond A Frizzell Bruce A Stanton

BACKGROUND Serum- and glucocorticoid-inducible kinase-1 (SGK1) increases CFTR Cl currents in Xenopus oocytes by an unknown mechanism. Because SGK increases the plasma membrane expression of other ion channels, the goal of this paper was to test the hypothesis that SGK1 stimulates CFTR Cl currents by increasing the number of CFTR Cl channels in the plasma membrane. METHODS CFTR Cl currents wer...

Journal: :Proceedings of the National Academy of Sciences of the United States of America 1997
G B Pier M Grout T S Zaidi

The cystic fibrosis transmembrane conductance regulator (CFTR) is a chloride ion channel, but its relationship to the primary clinical manifestation of CF, chronic Pseudomonas aeruginosa pulmonary infection, is unclear. We report that CFTR is a cellular receptor for binding, endocytosing, and clearing P. aeruginosa from the normal lung. Murine cells expressing recombinant human wild-type CFTR i...

2011
Loïc Drévillon Gaëlle Tanguy Alexandre Hinzpeter Nicole Arous Alix de Becdelièvre Abdel Aissat Agathe Tarze Michel Goossens Pascale Fanen

The CFTR (cystic fibrosis transmembrane conductance regulator) protein is a large polytopic protein whose biogenesis is inefficient. To better understand the regulation of CFTR processing and trafficking, we conducted a genetic screen that identified COMMD1 as a new CFTR partner. COMMD1 is a protein associated with multiple cellular pathways, including the regulation of hepatic copper excretion...

2014
Jennifer M. Bomberger Bonita A. Coutermarsh Roxanna L. Barnaby J. Denry Sato M. Christine Chapline Bruce A. Stanton

BACKGROUND Chloride (Cl) secretion by the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) located in the apical membrane of respiratory epithelial cells plays a critical role in maintenance of the airway surface liquid and mucociliary clearance of pathogens. Previously, we and others have shown that the serum and glucocorticoid-inducible kinase-1 (SGK1) increases wild type CFTR (wt-C...

2013
Jie Cheng William Guggino

Golgi-localized cystic fibrosis transmembrane conductance regulator (CFTR)-associated ligand (CAL) and syntaxin 6 (STX6) regulate the abundance of mature, post-ER CFTR by forming a CAL/STX6/CFTR complex (CAL complex) that promotes CFTR degradation in lysosomes. However, the molecular mechanism underlying this degradation is unknown. Here we investigated the interaction of a Golgi-localized, mem...

Journal: :Cell 2000
Shusheng Wang Hongwen Yue Rachel B Derin William B Guggino Min Li

The cystic fibrosis transmembrane conductance regulator (CFTR) gene encodes a chloride channel protein that belongs to the superfamily of ATP binding cassette (ABC) transporters. Phosphorylation by protein kinase A in the presence of ATP activates the CFTR-mediated chloride conductance of the apical membranes. We have identified a novel hydrophilic CFTR binding protein, CAP70, which is also con...

Journal: :Molecular medicine reports 2016
Jiao Xu Liangbo Lin Min Yong Xiaojing Dong Tinghe Yu Lina Hu

The cystic fibrosis transmembrane conductance regulator (CFTR) belongs to the adenosine triphosphate‑binding cassette transporter family, members of which are involved in several types of cancer. Previous studies by our group reported that CFTR was highly expressed in serous ovarian cancer (SOC) tissues, and that knockdown of CFTR suppressed the proliferation of ovarian cancer in vitro and in v...

Journal: :Biochemical and biophysical research communications 2007
Kazutsune Harada Tsukasa Okiyoneda Yasuaki Hashimoto Kimiko Oyokawa Kimitoshi Nakamura Mary Ann Suico Tsuyoshi Shuto Hirofumi Kai

Curcumin has been reported to correct cystic fibrosis caused by the DeltaF508 mutation of the cystic fibrosis transmembrane regulator (CFTR) but its mechanistic action remains unclear. We have recently demonstrated that the ER chaperone calreticulin (CRT) negatively regulates the CFTR cell surface expression and activity. Thus, we aimed at determining whether CRT mediates the effect of curcumin...

Journal: :Cell 2017
Fangyu Liu Zhe Zhang László Csanády David C. Gadsby Jue Chen

The cystic fibrosis transmembrane conductance regulator (CFTR) is an ATP-binding cassette (ABC) transporter that uniquely functions as an ion channel. Here, we present a 3.9 Å structure of dephosphorylated human CFTR without nucleotides, determined by electron cryomicroscopy (cryo-EM). Close resemblance of this human CFTR structure to zebrafish CFTR under identical conditions reinforces its rel...

Journal: :Cellular physiology and biochemistry : international journal of experimental cellular physiology, biochemistry, and pharmacology 2012
Hiromi Uramoto Toshiaki Okada Yasunobu Okada

BACKGROUND The cardiac isoform of the cystic fibrosis transmembrane conductance regulator (CFTR) was shown to be activated by β-adrenergic or purinergic stimulation and involved in cell volume regulation after osmotic swelling. Also, cardiac CFTR was reported to be essential in the mechanism by which ischemic preconditioning protects against ischemia/reperfusion(I/ R)-induced injury of the hear...

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