Journal articles on the topic 'CFTR function'
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Bossard, Florian, Amal Robay, Gilles Toumaniantz, Shehrazade Dahimene, Frédéric Becq, Jean Merot, and Chantal Gauthier. "NHE-RF1 protein rescues ΔF508-CFTR function." American Journal of Physiology-Lung Cellular and Molecular Physiology 292, no. 5 (May 2007): L1085—L1094. http://dx.doi.org/10.1152/ajplung.00445.2005.
Full textBorkenhagen, Beatrice, and Peter Prehm. "Recovery of ΔF508-CFTR Function by Citrate." Nutrients 14, no. 20 (October 14, 2022): 4283. http://dx.doi.org/10.3390/nu14204283.
Full textBRADBURY, NEIL A. "Intracellular CFTR: Localization and Function." Physiological Reviews 79, no. 1 (January 1, 1999): S175—S191. http://dx.doi.org/10.1152/physrev.1999.79.1.s175.
Full textCarroll, Tiziana Piazza, Erik M. Schwiebert, and William B. Guggino. "CFTR: Structure and Function." Cellular Physiology and Biochemistry 3, no. 5-6 (1993): 388–99. http://dx.doi.org/10.1159/000154700.
Full textMeng, Xin, Jack Clews, Anca D. Ciuta, Eleanor R. Martin, and Robert C. Ford. "CFTR structure, stability, function and regulation." Biological Chemistry 400, no. 10 (October 25, 2019): 1359–70. http://dx.doi.org/10.1515/hsz-2018-0470.
Full textRamalho, Anabela S., Eva Fürstová, Annelotte M. Vonk, Marc Ferrante, Catherine Verfaillie, Lieven Dupont, Mieke Boon, et al. "Correction of CFTR function in intestinal organoids to guide treatment of cystic fibrosis." European Respiratory Journal 57, no. 1 (August 3, 2020): 1902426. http://dx.doi.org/10.1183/13993003.02426-2019.
Full textLondino, James D., Ahmed Lazrak, Asta Jurkuvenaite, James F. Collawn, James W. Noah, and Sadis Matalon. "Influenza matrix protein 2 alters CFTR expression and function through its ion channel activity." American Journal of Physiology-Lung Cellular and Molecular Physiology 304, no. 9 (May 1, 2013): L582—L592. http://dx.doi.org/10.1152/ajplung.00314.2012.
Full textTaylor-Cousar, Jennifer L., Marcus A. Mall, Bonnie W. Ramsey, Edward F. McKone, Elizabeth Tullis, Gautham Marigowda, Charlotte M. McKee, et al. "Clinical development of triple-combination CFTR modulators for cystic fibrosis patients with one or two F508del alleles." ERJ Open Research 5, no. 2 (April 2019): 00082–2019. http://dx.doi.org/10.1183/23120541.00082-2019.
Full textMaitra, Rangan, Perumal Sivashanmugam, and Keith Warner. "A Rapid Membrane Potential Assay to Monitor CFTR Function and Inhibition." Journal of Biomolecular Screening 18, no. 9 (May 7, 2013): 1132–37. http://dx.doi.org/10.1177/1087057113488420.
Full textBertrand, Carol A., and Raymond A. Frizzell. "The role of regulated CFTR trafficking in epithelial secretion." American Journal of Physiology-Cell Physiology 285, no. 1 (July 2003): C1—C18. http://dx.doi.org/10.1152/ajpcell.00554.2002.
Full textRamalho, Anabela S., Mieke Boon, Marijke Proesmans, François Vermeulen, Marianne S. Carlon, and Kris Boeck. "Assays of CFTR Function In Vitro, Ex Vivo and In Vivo." International Journal of Molecular Sciences 23, no. 3 (January 27, 2022): 1437. http://dx.doi.org/10.3390/ijms23031437.
Full textNiedermayr, Katharina, Verena Gasser, Claudia Rueckes-Nilges, Dorothea Appelt, Johannes Eder, Teresa Fuchs, Lutz Naehrlich, and Helmut Ellemunter. "Personalized medicine with drugs targeting the underlying protein defect in cystic fibrosis: is monitoring of treatment response necessary?" Therapeutic Advances in Chronic Disease 13 (January 2022): 204062232211086. http://dx.doi.org/10.1177/20406223221108627.
Full textSouza-Menezes, Jackson, Geórgia da Silva Feltran, and Marcelo M. Morales. "CFTR and TNR-CFTR expression and function in the kidney." Biophysical Reviews 6, no. 2 (May 7, 2014): 227–36. http://dx.doi.org/10.1007/s12551-014-0140-8.
Full textBengtson, Charles, Neerupma Silswal, Nathalie Baumlin, Makoto Yoshida, John Dennis, Sireesha Yerrathota, Michael Kim, and Matthias Salathe. "The CFTR Amplifier Nesolicaftor Rescues TGF-β1 Inhibition of Modulator-Corrected F508del CFTR Function." International Journal of Molecular Sciences 23, no. 18 (September 19, 2022): 10956. http://dx.doi.org/10.3390/ijms231810956.
Full textValdivieso, Angel Gabriel, and Tomás A. Santa-Coloma. "CFTR activity and mitochondrial function." Redox Biology 1, no. 1 (2013): 190–202. http://dx.doi.org/10.1016/j.redox.2012.11.007.
Full textBitam, Sara, Iwona Pranke, Monika Hollenhorst, Nathalie Servel, Christelle Moquereau, Danielle Tondelier, Aurélie Hatton, et al. "An unexpected effect of TNF-α on F508del-CFTR maturation and function." F1000Research 4 (July 10, 2015): 218. http://dx.doi.org/10.12688/f1000research.6683.1.
Full textBitam, Sara, Iwona Pranke, Monika Hollenhorst, Nathalie Servel, Christelle Moquereau, Danielle Tondelier, Aurélie Hatton, et al. "An unexpected effect of TNF-α on F508del-CFTR maturation and function." F1000Research 4 (September 2, 2015): 218. http://dx.doi.org/10.12688/f1000research.6683.2.
Full textBlackledge, Neil P., Emma J. Carter, Joanne R. Evans, Victoria Lawson, Rebecca K. Rowntree, and Ann Harris. "CTCF mediates insulator function at the CFTR locus." Biochemical Journal 408, no. 2 (November 14, 2007): 267–75. http://dx.doi.org/10.1042/bj20070429.
Full textBarrio, Raquel. "MANAGEMENT OF ENDOCRINE DISEASE: Cystic fibrosis-related diabetes: novel pathogenic insights opening new therapeutic avenues." European Journal of Endocrinology 172, no. 4 (April 2015): R131—R141. http://dx.doi.org/10.1530/eje-14-0644.
Full textCao, Huibi, Hong Ouyang, Onofrio Laselva, Claire Bartlett, Zhichang Peter Zhou, Cathleen Duan, Tarini Gunawardena, et al. "A helper-dependent adenoviral vector rescues CFTR to wild-type functional levels in cystic fibrosis epithelial cells harbouring class I mutations." European Respiratory Journal 56, no. 5 (May 26, 2020): 2000205. http://dx.doi.org/10.1183/13993003.00205-2020.
Full textSCHWIEBERT, ERIK M., DALE J. BENOS, MARIE E. EGAN, M. JACKSON STUTTS, and WILLIAM B. GUGGINO. "CFTR Is a Conductance Regulator as well as a Chloride Channel." Physiological Reviews 79, no. 1 (January 1, 1999): S145—S166. http://dx.doi.org/10.1152/physrev.1999.79.1.s145.
Full textBacalhau, Mafalda, Filipa C. Ferreira, Iris A. L. Silva, Camilla D. Buarque, Margarida D. Amaral, and Miquéias Lopes-Pacheco. "Additive Potentiation of R334W-CFTR Function by Novel Small Molecules." Journal of Personalized Medicine 13, no. 1 (January 1, 2023): 102. http://dx.doi.org/10.3390/jpm13010102.
Full textAlcolado, Nicole G., Dustin J. Conrad, Diogo Poroca, Mansong Li, Walaa Alshafie, Frederic G. Chappe, Ryan M. Pelis, et al. "Cystic fibrosis transmembrane conductance regulator dysfunction in VIP knockout mice." American Journal of Physiology-Cell Physiology 307, no. 2 (July 15, 2014): C195—C207. http://dx.doi.org/10.1152/ajpcell.00293.2013.
Full textSHEPPARD, DAVID N., and MICHAEL J. WELSH. "Structure and Function of the CFTR Chloride Channel." Physiological Reviews 79, no. 1 (January 1, 1999): S23—S45. http://dx.doi.org/10.1152/physrev.1999.79.1.s23.
Full textLotti, Virginia, Flavia Merigo, Anna Lagni, Andrea Di Clemente, Marco Ligozzi, Paolo Bernardi, Giada Rossini, et al. "CFTR Modulation Reduces SARS-CoV-2 Infection in Human Bronchial Epithelial Cells." Cells 11, no. 8 (April 15, 2022): 1347. http://dx.doi.org/10.3390/cells11081347.
Full textTrinh, Nguyen Thu Ngan, Claudia Bilodeau, Émilie Maillé, Manon Ruffin, Marie-Claude Quintal, Martin-Yvon Desrosiers, Simon Rousseau, and Emmanuelle Brochiero. "Deleterious impact of Pseudomonas aeruginosa on cystic fibrosis transmembrane conductance regulator function and rescue in airway epithelial cells." European Respiratory Journal 45, no. 6 (March 18, 2015): 1590–602. http://dx.doi.org/10.1183/09031936.00076214.
Full textSilva, Iris A. L., Violeta Railean, Aires Duarte, and Margarida D. Amaral. "Personalized Medicine Based on Nasal Epithelial Cells: Comparative Studies with Rectal Biopsies and Intestinal Organoids." Journal of Personalized Medicine 11, no. 5 (May 16, 2021): 421. http://dx.doi.org/10.3390/jpm11050421.
Full textHinata, Daichi, Ryosuke Fukuda, and Tsukasa Okiyoneda. "The COPD-Associated Polymorphism Impairs the CFTR Function to Suppress Excessive IL-8 Production upon Environmental Pathogen Exposure." International Journal of Molecular Sciences 24, no. 3 (January 24, 2023): 2305. http://dx.doi.org/10.3390/ijms24032305.
Full textBose, Samuel J., Toby S. Scott-Ward, Zhiwei Cai, and David N. Sheppard. "Exploiting species differences to understand the CFTR Cl− channel." Biochemical Society Transactions 43, no. 5 (October 1, 2015): 975–82. http://dx.doi.org/10.1042/bst20150129.
Full textRen, Hong Yu, Diane E. Grove, Oxana De La Rosa, Scott A. Houck, Pattarawut Sopha, Fredrick Van Goor, Beth J. Hoffman, and Douglas M. Cyr. "VX-809 corrects folding defects in cystic fibrosis transmembrane conductance regulator protein through action on membrane-spanning domain 1." Molecular Biology of the Cell 24, no. 19 (October 2013): 3016–24. http://dx.doi.org/10.1091/mbc.e13-05-0240.
Full textGraeber, Simon Y., Constanze Vitzthum, and Marcus A. Mall. "Potential of Intestinal Current Measurement for Personalized Treatment of Patients with Cystic Fibrosis." Journal of Personalized Medicine 11, no. 5 (May 8, 2021): 384. http://dx.doi.org/10.3390/jpm11050384.
Full textSmith, Stephen S., Xuehong Liu, Zhi-Ren Zhang, Fang Sun, Thomas E. Kriewall, Nael A. McCarty, and David C. Dawson. "Cftr." Journal of General Physiology 118, no. 4 (October 1, 2001): 407–32. http://dx.doi.org/10.1085/jgp.118.4.407.
Full textFiedorczuk, Karol, and Jue Chen. "Molecular structures reveal synergistic rescue of Δ508 CFTR by Trikafta modulators." Science 378, no. 6617 (October 21, 2022): 284–90. http://dx.doi.org/10.1126/science.ade2216.
Full textBaldassarri, Margherita, Kristina Zguro, Valeria Tomati, Cristina Pastorino, Francesca Fava, Susanna Croci, Mirella Bruttini, et al. "Gain- and Loss-of-Function CFTR Alleles Are Associated with COVID-19 Clinical Outcomes." Cells 11, no. 24 (December 16, 2022): 4096. http://dx.doi.org/10.3390/cells11244096.
Full textVoronkova, A. Yu, E. I. Kondratyeva, E. D. Nikolaeva, E. V. Pasnova, I. R. Fatkhullina, and N. D. Odinaeva. "Pancreatitis in children with cystic fibrosis and preserved pancreatic exocrine function." Voprosy praktičeskoj pediatrii 17, no. 3 (2022): 120–27. http://dx.doi.org/10.20953/1817-7646-2022-3-120-127.
Full textWang, Guoshun, and Hang Pong Ng. "Myeloid CFTR Loss-of-function Causes Persistent Neutrophilic Inflammation in Cystic Fibrosis." Journal of Immunology 202, no. 1_Supplement (May 1, 2019): 187.33. http://dx.doi.org/10.4049/jimmunol.202.supp.187.33.
Full textPecho-Silva, Samuel, and Ana C. Navarro-Solsol. "The c.3274T> C mutation in the CFTR gene results in bronchiectasis and loss of lung function in a 44-year-old Peruvian woman: A very rare condition." Revista Peruana de Investigación en Salud 5, no. 2 (April 9, 2021): 132–35. http://dx.doi.org/10.35839/repis.5.2.1008.
Full textVan Goor, F., H. Yu, B. Burton, C. J. Huang, and B. J. Hoffman. "WS14.1 Ivacaftor potentiates mutant CFTR forms associated with residual CFTR function." Journal of Cystic Fibrosis 11 (June 2012): S31. http://dx.doi.org/10.1016/s1569-1993(12)60097-6.
Full textShi, Juan, Hui Li, Chao Yuan, Meihui Luo, Jun Wei, and Xiaoming Liu. "Cigarette Smoke-Induced Acquired Dysfunction of Cystic Fibrosis Transmembrane Conductance Regulator in the Pathogenesis of Chronic Obstructive Pulmonary Disease." Oxidative Medicine and Cellular Longevity 2018 (2018): 1–13. http://dx.doi.org/10.1155/2018/6567578.
Full textWestholm, Efraim, Anna Wendt, and Lena Eliasson. "Islet Function in the Pathogenesis of Cystic Fibrosis-Related Diabetes Mellitus." Clinical Medicine Insights: Endocrinology and Diabetes 14 (January 2021): 117955142110312. http://dx.doi.org/10.1177/11795514211031204.
Full textDegrugillier, Fanny, Abdel Aissat, Virginie Prulière-Escabasse, Lucie Bizard, Benjamin Simonneau, Xavier Decrouy, Chong Jiang, Daniela Rotin, Pascale Fanen, and Stéphanie Simon. "Phosphorylation of the Chaperone-Like HspB5 Rescues Trafficking and Function of F508del-CFTR." International Journal of Molecular Sciences 21, no. 14 (July 8, 2020): 4844. http://dx.doi.org/10.3390/ijms21144844.
Full textHwang, Tzyh-Chang, Jiunn-Tyng Yeh, Jingyao Zhang, Ying-Chun Yu, Han-I. Yeh, and Samantha Destefano. "Structural mechanisms of CFTR function and dysfunction." Journal of General Physiology 150, no. 4 (March 26, 2018): 539–70. http://dx.doi.org/10.1085/jgp.201711946.
Full textPrins, Stella, Emily Langron, Cato Hastings, Emily J. Hill, Andra C. Stefan, Lewis D. Griffin, and Paola Vergani. "Fluorescence assay for simultaneous quantification of CFTR ion-channel function and plasma membrane proximity." Journal of Biological Chemistry 295, no. 49 (September 15, 2020): 16529–44. http://dx.doi.org/10.1074/jbc.ra120.014061.
Full textBai, Yonghong, Min Li, and Tzyh-Chang Hwang. "Structural basis for the channel function of a degraded ABC transporter, CFTR (ABCC7)." Journal of General Physiology 138, no. 5 (October 31, 2011): 495–507. http://dx.doi.org/10.1085/jgp.201110705.
Full textWeber, Adam J., Grace Soong, Ruth Bryan, Shahryar Saba, and Alice Prince. "Activation of NF-κB in airway epithelial cells is dependent on CFTR trafficking and Cl− channel function." American Journal of Physiology-Lung Cellular and Molecular Physiology 281, no. 1 (July 1, 2001): L71—L78. http://dx.doi.org/10.1152/ajplung.2001.281.1.l71.
Full textFuller, C. M., and D. J. Benos. "CFTR!" American Journal of Physiology-Cell Physiology 263, no. 2 (August 1, 1992): C267—C286. http://dx.doi.org/10.1152/ajpcell.1992.263.2.c267.
Full textAngyal, Dora, Marcel J. C. Bijvelds, Marco J. Bruno, Maikel P. Peppelenbosch, and Hugo R. de Jonge. "Bicarbonate Transport in Cystic Fibrosis and Pancreatitis." Cells 11, no. 1 (December 24, 2021): 54. http://dx.doi.org/10.3390/cells11010054.
Full textUliyakina, Inna, Hugo M. Botelho, Ana C. da Paula, Sara Afonso, Miguel J. Lobo, Verónica Felício, Carlos M. Farinha, and Margarida D. Amaral. "Full Rescue of F508del-CFTR Processing and Function by CFTR Modulators Can Be Achieved by Removal of Two Regulatory Regions." International Journal of Molecular Sciences 21, no. 12 (June 25, 2020): 4524. http://dx.doi.org/10.3390/ijms21124524.
Full textSousa, Luis, Ines Pankonien, Luka A. Clarke, Iris Silva, Karl Kunzelmann, and Margarida D. Amaral. "KLF4 Acts as a wt-CFTR Suppressor through an AKT-Mediated Pathway." Cells 9, no. 7 (July 2, 2020): 1607. http://dx.doi.org/10.3390/cells9071607.
Full textShin, Yonghwan, Minkyoung Kim, Jonghwa Won, Junchul Kim, Seog Bae Oh, Jong-Ho Lee, and Kyungpyo Park. "Epigenetic Modification of CFTR in Head and Neck Cancer." Journal of Clinical Medicine 9, no. 3 (March 9, 2020): 734. http://dx.doi.org/10.3390/jcm9030734.
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