نتایج جستجو برای: ژن cftr

تعداد نتایج: 21533  

Journal: :American journal of physiology. Cell physiology 2000
M Howard X Jiang D B Stolz W G Hill J A Johnson S C Watkins R A Frizzell C M Bruton P D Robbins O A Weisz

Channel gating of the cystic fibrosis transmembrane conductance regulator (CFTR) is activated in response to cAMP stimulation. In addition, CFTR activation may also involve rapid insertion of a subapical pool of CFTR into the plasma membrane (PM). However, this issue has been controversial, in part because of the difficulty in distinguishing cell surface vs. intracellular CFTR. Recently, a full...

2010
Himabindu Penmatsa Weiqiang Zhang Sunitha Yarlagadda Chunying Li Veronica G. Conoley Junming Yue Suleiman W. Bahouth Randal K. Buddington Guangping Zhang Deborah J. Nelson Monal D. Sonecha Vincent Manganiello Jeffrey J. Wine Anjaparavanda P. Naren

Formation of multiple-protein macromolecular complexes at specialized subcellular microdomains increases the specificity and efficiency of signaling in cells. In this study, we demonstrate that phosphodiesterase type 3A (PDE3A) physically and functionally interacts with cystic fibrosis transmembrane conductance regulator (CFTR) channel. PDE3A inhibition generates compartmentalized cyclic adenos...

Journal: :Infection and immunity 2002
Jeffrey B Lyczak Gerald B Pier

The cystic fibrosis transmembrane conductance regulator (CFTR) protein is an epithelial receptor mediating the translocation of Salmonella enterica serovar Typhi to the gastric submucosa. Since the level of cell surface CFTR is directly related to the efficiency of serovar Typhi translocation, the goal of this study was to measure CFTR expression by the intestinal epithelium during infection. C...

Journal: :JOP : Journal of the pancreas 2001
M M Reddy P M Quinton

While cystic fibrosis transmembrane conductance regulator (CFTR) is well known to function as a Cl(-) channel, some mutations in the channel protein causing cystic fibrosis (CF) disrupt another vital physiological function, HCO(3)(-) transport. Pathological implications of derailed HCO(3)(-) transport are clearly demonstrated by the pancreatic destruction that accompany certain mutations in CF....

Journal: :Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society 2011
C Bombieri M Claustres K De Boeck N Derichs J Dodge E Girodon I Sermet M Schwarz M Tzetis M Wilschanski C Bareil D Bilton C Castellani H Cuppens G R Cutting P Drevínek P Farrell J S Elborn K Jarvi B Kerem E Kerem M Knowles M Macek A Munck D Radojkovic M Seia D N Sheppard K W Southern M Stuhrmann E Tullis J Zielenski P F Pignatti C Ferec

Several diseases have been clinically or genetically related to cystic fibrosis (CF), but a consensus definition is lacking. Here, we present a proposal for consensus guidelines on cystic fibrosis transmembrane conductance regulator (CFTR)-related disorders (CFTR-RDs), reached after expert discussion and two dedicated workshops. A CFTR-RD may be defined as "a clinical entity associated with CFT...

Journal: :Proceedings of the National Academy of Sciences of the United States of America 2006
Emanuela M Bruscia Joanna E Price Ee-Chun Cheng Scott Weiner Christina Caputo Elisa C Ferreira Marie E Egan Diane S Krause

Several studies have demonstrated that bone marrow (BM)-derived cells give rise to rare epithelial cells in the gastrointestinal (GI) and respiratory tracts after BM transplantation into myeloablated recipients. We investigate whether, after transplantation of cystic fibrosis transmembrane conductance regulator (CFTR)-positive BM-derived cells, BM-derived GI and airway epithelial cells can prov...

Journal: :Journal of visualized experiments : JoVE 2012
Yanning Wu Shuo Wang Chunying Li

Cystic fibrosis transmembrane conductance regulator (CFTR), a chloride channel located primarily at the apical membranes of epithelial cells, plays a crucial role in transepithelial fluid homeostasis(1-3). CFTR has been implicated in two major diseases: cystic fibrosis (CF)(4) and secretory diarrhea(5). In CF, the synthesis or functional activity of the CFTR Cl- channel is reduced. This disorde...

2011
Luc Dannhoffer Arnaud Billet Mathilde Jollivet Patricia Melin-Heschel Christelle Faveau Frédéric Becq

Cystic fibrosis (CF) is a major inherited disorder involving abnormalities of fluid and electrolyte transport in a number of different organs due to abnormal function of cystic fibrosis transmembrane conductance regulator (CFTR) protein. We recently identified a family of CFTR activators, which contains the hit: RP107 [7-n-butyl-6-(4-hydroxyphenyl)[5H]-pyrrolo[2,3-b]pyrazine]. Here, we further ...

Journal: :The Journal of clinical investigation 2002
Tonghui Ma Jay R Thiagarajah Hong Yang Nitin D Sonawane Chiara Folli Luis J V Galietta A S Verkman

Secretory diarrhea is the leading cause of infant death in developing countries and a major cause of morbidity in adults. The cystic fibrosis transmembrane conductance regulator (CFTR) protein is required for fluid secretion in the intestine and airways and, when defective, causes the lethal genetic disease cystic fibrosis. We screened 50,000 chemically diverse compounds for inhibition of cAMP/...

2016
Ziwei Tu Qu Chen Jie Ting Zhang Xiaohua Jiang Yunfei Xia Hsiao Chang Chan

While there is an increasing interest in the correlation of cystic fibrosis transmembrane conductance regulator (CFTR) and cancer incidence, the role of CFTR in nasopharyngeal carcinoma (NPC) development remains unknown. In this study, we aimed to explore the prognostic value of CFTR in NPC patients. The expression of CFTR was determined in NPC cell lines and tissues. Statistical analysis was u...

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