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233 results for Cystic+Fibrosis+Transmembrane+Conductance+Regulator+-+physiology

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ArticlesPeer ReviewedFull Text
Impaired mucus detachment disrupts mucociliary transport in a piglet model of cystic fibrosis
Author / Creator:Hoegger, Mark J. ; Fischer, Anthony J. ; McMenimen, James D. ; Ostedgaard, Lynda S. ; Tucker, Alex J. ; Awadalla, Maged A. ; Moninger, Thomas O. ; Michalski, Andrew S. ; Hoffman, Eric A. ; Zabner, Joseph ; Stoltz, David A. ; Welsh, Michael J.
Publisher:United States: American Association for the Advancement of Science
Journal title:Science (American Association for the Advancement of Science), 2014-08, Vol.345 (6198), p.818-822
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Mechanism-based corrector combination restores ΔF508-CFTR folding and function
Author / Creator:Okiyoneda, Tsukasa ; Veit, Guido ; Dekkers, Johanna F ; Bagdany, Miklos ; Soya, Naoto ; Xu, Haijin ; Roldan, Ariel ; Verkman, Alan S ; Kurth, Mark ; Simon, Agnes ; Hegedus, Tamas ; Beekman, Jeffrey M ; Lukacs, Gergely L
Publisher:New York: Nature Publishing Group US
Journal title:Nature chemical biology, 2013-07, Vol.9 (7), p.444-454
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Correlating Cystic Fibrosis Transmembrane Conductance Regulator Function with Clinical Features to Inform Precision Treatment of Cystic Fibrosis
Author / Creator:McCague, Allison F. ; Raraigh, Karen S. ; Pellicore, Matthew J. ; Davis-Marcisak, Emily F. ; Evans, Taylor A. ; Han, Sangwoo T. ; Lu, Zhongzhou ; Joynt, Anya T. ; Sharma, Neeraj ; Castellani, Carlo ; Collaco, Joseph M. ; Corey, Mary ; Lewis, Michelle H. ; Penland, Chris M. ; Rommens, Johanna M. ; Stephenson, Anne L. ; Sosnay, Patrick R. ; Cutting, Garry R.
Publisher:United States: American Thoracic Society
Journal title:American journal of respiratory and critical care medicine, 2019-05, Vol.199 (9), p.1116-1126
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Pseudomonas aeruginosa LasB protease impairs innate immunity in mice and humans by targeting a lung epithelial cystic fibrosis transmembrane regulator–IL-6–antimicrobial–repair pathway
Author / Creator:Saint-Criq, Vinciane ; Villeret, Bérengère ; Bastaert, Fabien ; Kheir, Saadé ; Hatton, Aurélie ; Cazes, Aurélie ; Xing, Zhou ; Sermet-Gaudelus, Isabelle ; Garcia-Verdugo, Ignacio ; Edelman, Aleksander ; Sallenave, Jean-Michel
Publisher:England: BMJ Publishing Group LTD
Journal title:Thorax, 2018-01, Vol.73 (1), p.49-61
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Cystic fibrosis
Author / Creator:O'Sullivan, Brian P, Prof ; Freedman, Steven D, MD
Publisher:Kidlington: Elsevier Ltd
Journal title:The Lancet (British edition), 2009-05, Vol.373 (9678), p.1891-1904
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DNAJB12 and Hsp70 triage arrested intermediates of N1303K-CFTR for endoplasmic reticulum-associated autophagy
Author / Creator:He, Lihua ; Kennedy, Andrew S. ; Houck, Scott ; Aleksandrov, Andrei ; Quinney, Nancy L. ; Cyr-Scully, Alexandra ; Cholon, Deborah M. ; Gentzsch, Martina ; Randell, Scott H. ; Ren, Hong Yu ; Cyr, Douglas M.
Publisher:United States: The American Society for Cell Biology
Journal title:Molecular biology of the cell, 2021-04, Vol.32 (7), p.538-553
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Cystic fibrosis from the gastroenterologist's perspective
Author / Creator:Ooi, Chee Y. ; Durie, Peter R.
Publisher:London: Nature Publishing Group UK
Journal title:Nature reviews. Gastroenterology & hepatology, 2016-03, Vol.13 (3), p.175-185
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Chloride channels as drug targets
Author / Creator:Verkman, Alan S. ; Galietta, Luis J. V.
Publisher:London: Nature Publishing Group UK
Journal title:Nature reviews. Drug discovery, 2009-02, Vol.8 (2), p.153-171
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Cystic Fibrosis Transmembrane Conductance Regulator in Sarcoplasmic Reticulum of Airway Smooth Muscle. Implications for Airway Contractility
Author / Creator:Cook, Daniel P. ; Rector, Michael V. ; Bouzek, Drake C. ; Michalski, Andrew S. ; Gansemer, Nicholas D. ; Reznikov, Leah R. ; Li, Xiaopeng ; Stroik, Mallory R. ; Ostedgaard, Lynda S. ; Abou Alaiwa, Mahmoud H. ; Thompson, Michael A. ; Prakash, Y. S. ; Krishnan, Ramaswamy ; Meyerholz, David K. ; Seow, Chun Y. ; Stoltz, David A.
Publisher:United States: American Thoracic Society
Journal title:American journal of respiratory and critical care medicine, 2016-02, Vol.193 (4), p.417-426
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Anoctamin 6 is an essential component of the outwardly rectifying chloride channel
Author / Creator:Martins, Joana Raquel ; Faria, Diana ; Kongsuphol, Patthara ; Reisch, Barbara ; Schreiber, Rainer ; Kunzelmann, Karl
Publisher:United States: National Academy of Sciences
Journal title:Proceedings of the National Academy of Sciences - PNAS, 2011-11, Vol.108 (44), p.18168-18172
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Phenylalanine-508 mediates a cytoplasmic-membrane domain contact in the CFTR 3D structure crucial to assembly and channel function
Author / Creator:Serohijos, Adrian W.R ; Hegedűs, Tamás ; Aleksandrov, Andrei A ; He, Lihua ; Cui, Liying ; Dokholyan, Nikolay V ; Riordan, John R
Publisher:United States: National Academy of Sciences
Journal title:Proceedings of the National Academy of Sciences - PNAS, 2008-03, Vol.105 (9), p.3256-3261
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Alkalosis-induced hypoventilation in cystic fibrosis: The importance of efficient renal adaptation
Author / Creator:Berg, Peder ; Andersen, Jesper Frank ; Sørensen, Mads Vaarby ; Wang, Tobias ; Malte, Hans ; Leipziger, Jens
Publisher:United States: National Academy of Sciences
Journal title:Proceedings of the National Academy of Sciences - PNAS, 2022-02, Vol.119 (8), p.1-7
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Ataluren (PTC124) Induces Cystic Fibrosis Transmembrane Conductance Regulator Protein Expression and Activity in Children with Nonsense Mutation Cystic Fibrosis
Author / Creator:Sermet-Gaudelus, Isabelle ; Boeck, Kris De ; Casimir, Georges J. ; Vermeulen, François ; Leal, Teresinha ; Mogenet, Agnès ; Roussel, Delphine ; Fritsch, Janine ; Hanssens, Laurence ; Hirawat, Samit ; Miller, Nilsen L. ; Constantine, Scott ; Reha, Allen ; Ajayi, Temitayo ; Elfring, Gary L. ; Miller, Langdon L.
Publisher:New York, NY: American Thoracic Society
Journal title:American journal of respiratory and critical care medicine, 2010-11, Vol.182 (10), p.1262-1272
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CFTR regulates B cell activation and lymphoid follicle development
Author / Creator:Polverino, Francesca ; Lu, Bao ; Quintero, Joselyn Rojas ; Vargas, Sara O. ; Patel, Avignat S. ; Owen, Caroline A. ; Gerard, Norma P. ; Gerard, Craig ; Cernadas, Manuela
Publisher:England: BioMed Central Ltd
Journal title:Respiratory research, 2019-07, Vol.20 (1), p.133-133, Article 133
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Ion channels, phosphorylation and mammalian sperm capacitation
Author / Creator:Visconti, Pablo E ; Krapf, Dario ; de la Vega-Beltrán, José Luis ; Acevedo, Juan José ; Darszon, Alberto
Publisher:China: Medknow Publications & Media Pvt. Ltd
Journal title:Asian journal of andrology, 2011-05, Vol.13 (3), p.395-405
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Role of Sphingolipids in the Pathobiology of Lung Inflammation
Author / Creator:Ghidoni, Riccardo ; Signorelli, Paola ; Caretti, Anna
Publisher:Cairo, Egypt: Hindawi Limiteds
Journal title:Mediators of Inflammation, 2015-01, Vol.2015 (2015), p.413-431-238
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CFTR and Anoctamin 1 (ANO1) contribute to cAMP amplified exocytosis and insulin secretion in human and murine pancreatic beta-cells
Author / Creator:Edlund, Anna ; Esguerra, Jonathan LS ; Wendt, Anna ; Flodström-Tullberg, Malin ; Eliasson, Lena
Publisher:England: BioMed Central Ltd
Journal title:BMC medicine, 2014-05, Vol.12 (1), p.87-87, Article 87
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CFTR regulates phagosome acidification in macrophages and alters bactericidal activity
Author / Creator:Di, Anke ; Brown, Mary E. ; Deriy, Ludmila V. ; Li, Chunying ; Szeto, Frances L. ; Chen, Yimei ; Huang, Ping ; Tong, Jiankun ; Naren, Anjaparavanda P. ; Bindokas, Vytautas ; Palfrey, H. Clive ; Nelson, Deborah J.
Publisher:London: Nature Publishing Group UK
Journal title:Nature cell biology, 2006-09, Vol.8 (9), p.933-944
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Dysfunctional CFTR Alters the Bactericidal Activity of Human Macrophages against Pseudomonas aeruginosa
Author / Creator:Porto, Paola Del ; Cifani, Noemi ; Guarnieri, Simone ; Di Domenico, Enea Gino ; Mariggiò, Maria A. ; Spadaro, Francesca ; Guglietta, Silvia ; Anile, Marco ; Venuta, Federico ; Quattrucci, Serena ; Ascenzioni, Fiorentina
Publisher:United States: Public Library of Science
Journal title:PloS one, 2011-05, Vol.6 (5), p.e19970-e19970
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Altered iron metabolism in cystic fibrosis macrophages: the impact of CFTR modulators and implications for Pseudomonas aeruginosa survival
Author / Creator:Hazlett, H. F. ; Hampton, T. H. ; Aridgides, D. S. ; Armstrong, D. A. ; Dessaint, J. A. ; Mellinger, D. L. ; Nymon, A. B. ; Ashare, A.
Publisher:London: Nature Publishing Group UK
Journal title:Scientific reports, 2020-07, Vol.10 (1), p.10935, Article 10935
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Showing 1 to 20 of 233 results

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Impaired mucus detachment disrupts mucociliary transport in a piglet model of cystic fibrosis

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Mechanism-based corrector combination restores ΔF508-CFTR folding and function

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Correlating Cystic Fibrosis Transmembrane Conductance Regulator Function with Clinical Features to Inform Precision Treatment of Cystic Fibrosis

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Pseudomonas aeruginosa LasB protease impairs innate immunity in mice and humans by targeting a lung epithelial cystic fibrosis transmembrane regulator–IL-6–antimicrobial–repair pathway

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Cystic fibrosis

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DNAJB12 and Hsp70 triage arrested intermediates of N1303K-CFTR for endoplasmic reticulum-associated autophagy

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Cystic fibrosis from the gastroenterologist's perspective

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Chloride channels as drug targets

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Cystic Fibrosis Transmembrane Conductance Regulator in Sarcoplasmic Reticulum of Airway Smooth Muscle. Implications for Airway Contractility

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Anoctamin 6 is an essential component of the outwardly rectifying chloride channel

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Phenylalanine-508 mediates a cytoplasmic-membrane domain contact in the CFTR 3D structure crucial to assembly and channel function

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Alkalosis-induced hypoventilation in cystic fibrosis: The importance of efficient renal adaptation

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Ataluren (PTC124) Induces Cystic Fibrosis Transmembrane Conductance Regulator Protein Expression and Activity in Children with Nonsense Mutation Cystic Fibrosis

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CFTR regulates B cell activation and lymphoid follicle development

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Ion channels, phosphorylation and mammalian sperm capacitation

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Role of Sphingolipids in the Pathobiology of Lung Inflammation

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CFTR and Anoctamin 1 (ANO1) contribute to cAMP amplified exocytosis and insulin secretion in human and murine pancreatic beta-cells

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CFTR regulates phagosome acidification in macrophages and alters bactericidal activity

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Dysfunctional CFTR Alters the Bactericidal Activity of Human Macrophages against Pseudomonas aeruginosa

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Altered iron metabolism in cystic fibrosis macrophages: the impact of CFTR modulators and implications for Pseudomonas aeruginosa survival

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