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1.

A Therapy for Most with Cystic Fibrosis.

Bear, Christine E.

Cell ; 180(2): 211, 2020 01 23.

Artículo en Inglés | MEDLINE | ID: mdl-31978337

2.

Collagen Tubular Airway-on-Chip for Extended Epithelial Culture and Investigation of Ventilation Dynamics.

Gao, Wuyang; Kanagarajah, Kayshani R; Graham, Emma; Soon, Kayla; Veres, Teodor; Moraes, Theo J; Bear, Christine E; Veldhuizen, Ruud A; Wong, Amy P; Günther, Axel.

Small ; : e2309270, 2024 Mar 03.

Artículo en Inglés | MEDLINE | ID: mdl-38431940

3.

CFTR interactome mapping using the mammalian membrane two-hybrid high-throughput screening system.

Lim, Sang Hyun; Snider, Jamie; Birimberg-Schwartz, Liron; Ip, Wan; Serralha, Joana C; Botelho, Hugo M; Lopes-Pacheco, Miquéias; Pinto, Madalena C; Moutaoufik, Mohamed Taha; Zilocchi, Mara; Laselva, Onofrio; Esmaeili, Mohsen; Kotlyar, Max; Lyakisheva, Anna; Tang, Priscilla; López Vázquez, Lucía; Akula, Indira; Aboualizadeh, Farzaneh; Wong, Victoria; Grozavu, Ingrid; Opacak-Bernardi, Teuta; Yao, Zhong; Mendoza, Meg; Babu, Mohan; Jurisica, Igor; Gonska, Tanja; Bear, Christine E; Amaral, Margarida D; Stagljar, Igor.

Mol Syst Biol ; 18(2): e10629, 2022 02.

Artículo en Inglés | MEDLINE | ID: mdl-35156780

4.

A Cell-Based Optimised Approach for Rapid and Efficient Gene Editing of Human Pluripotent Stem Cells.

Cuevas-Ocaña, Sara; Yang, Jin Ye; Aushev, Magomet; Schlossmacher, George; Bear, Christine E; Hannan, Nicholas R F; Perkins, Neil D; Rossant, Janet; Wong, Amy P; Gray, Michael A.

Int J Mol Sci ; 24(12)2023 Jun 17.

Artículo en Inglés | MEDLINE | ID: mdl-37373413

5.

Rescue of multiple class II CFTR mutations by elexacaftor+tezacaftor+ivacaftor mediated in part by the dual activities of elexacaftor as both corrector and potentiator.

Laselva, Onofrio; Bartlett, Claire; Gunawardena, Tarini N A; Ouyang, Hong; Eckford, Paul D W; Moraes, Theo J; Bear, Christine E; Gonska, Tanja.

Eur Respir J ; 57(6)2021 06.

Artículo en Inglés | MEDLINE | ID: mdl-33303536

6.

A helper-dependent adenoviral vector rescues CFTR to wild-type functional levels in cystic fibrosis epithelial cells harbouring class I mutations.

Cao, Huibi; Ouyang, Hong; Laselva, Onofrio; Bartlett, Claire; Zhou, Zhichang Peter; Duan, Cathleen; Gunawardena, Tarini; Avolio, Julie; Bear, Christine E; Gonska, Tanja; Hu, Jim; Moraes, Theo J.

Eur Respir J ; 56(5)2020 11.

Artículo en Inglés | MEDLINE | ID: mdl-32457197

7.

Synergy of cAMP and calcium signaling pathways in CFTR regulation.

Bozoky, Zoltan; Ahmadi, Saumel; Milman, Tal; Kim, Tae Hun; Du, Kai; Di Paola, Michelle; Pasyk, Stan; Pekhletski, Roman; Keller, Jacob P; Bear, Christine E; Forman-Kay, Julie D.

Proc Natl Acad Sci U S A ; 114(11): E2086-E2095, 2017 03 14.

Artículo en Inglés | MEDLINE | ID: mdl-28242698

8.

Augmentation of Cystic Fibrosis Transmembrane Conductance Regulator Function in Human Bronchial Epithelial Cells via SLC6A14-Dependent Amino Acid Uptake. Implications for Treatment of Cystic Fibrosis.

Ahmadi, Saumel; Wu, Yu-Sheng; Li, Mingyuan; Ip, Wan; Lloyd-Kuzik, Andrew; Di Paola, Michelle; Du, Kai; Xia, Sunny; Lew, Alexandria; Bozoky, Zoltan; Forman-Kay, Julie; Bear, Christine E; Gonska, Tanja.

Am J Respir Cell Mol Biol ; 61(6): 755-764, 2019 12.

Artículo en Inglés | MEDLINE | ID: mdl-31189070

9.

ORKAMBI-Mediated Rescue of Mucociliary Clearance in Cystic Fibrosis Primary Respiratory Cultures Is Enhanced by Arginine Uptake, Arginase Inhibition, and Promotion of Nitric Oxide Signaling to the Cystic Fibrosis Transmembrane Conductance Regulator Channel.

Wu, Yu-Sheng; Jiang, Janet; Ahmadi, Saumel; Lew, Alexandria; Laselva, Onofrio; Xia, Sunny; Bartlett, Claire; Ip, Wan; Wellhauser, Leigh; Ouyang, Hong; Gonska, Tanja; Moraes, Theo J; Bear, Christine E.

Mol Pharmacol ; 96(4): 515-525, 2019 10.

Artículo en Inglés | MEDLINE | ID: mdl-31427400

10.

Correctors of the Major Cystic Fibrosis Mutant Interact through Membrane-Spanning Domains.

Laselva, Onofrio; Molinski, Steven; Casavola, Valeria; Bear, Christine E.

Mol Pharmacol ; 93(6): 612-618, 2018 06.

Artículo en Inglés | MEDLINE | ID: mdl-29618585

11.

Lipophilicity of the Cystic Fibrosis Drug, Ivacaftor (VX-770), and Its Destabilizing Effect on the Major CF-causing Mutation: F508del.

Chin, Stephanie; Hung, Maurita; Won, Amy; Wu, Yu-Sheng; Ahmadi, Saumel; Yang, Donghe; Elmallah, Salma; Toutah, Krimo; Hamilton, C Michael; Young, Robert N; Viirre, Russell D; Yip, Christopher M; Bear, Christine E.

Mol Pharmacol ; 94(2): 917-925, 2018 08.

Artículo en Inglés | MEDLINE | ID: mdl-29903751

12.

Attenuation of Phosphorylation-dependent Activation of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) by Disease-causing Mutations at the Transmission Interface.

Chin, Stephanie; Yang, Donghe; Miles, Andrew J; Eckford, Paul D W; Molinski, Steven; Wallace, B A; Bear, Christine E.

J Biol Chem ; 292(5): 1988-1999, 2017 02 03.

Artículo en Inglés | MEDLINE | ID: mdl-28003367

13.

Comprehensive mapping of cystic fibrosis mutations to CFTR protein identifies mutation clusters and molecular docking predicts corrector binding site.

Molinski, Steven V; Shahani, Vijay M; Subramanian, Adithya S; MacKinnon, Stephen S; Woollard, Geoffrey; Laforet, Marcon; Laselva, Onofrio; Morayniss, Leonard D; Bear, Christine E; Windemuth, Andreas.

Proteins ; 86(8): 833-843, 2018 08.

Artículo en Inglés | MEDLINE | ID: mdl-29569753

14.

Structural effects of extracellular loop mutations in CFTR helical hairpins.

Chang, Yuan-Heng; Stone, Tracy A; Chin, Stephanie; Glibowicka, Mira; Bear, Christine E; Deber, Charles M.

Biochim Biophys Acta Biomembr ; 1860(5): 1092-1098, 2018 May.

Artículo en Inglés | MEDLINE | ID: mdl-29307731

15.

Cystic fibrosis gene modifier SLC26A9 modulates airway response to CFTR-directed therapeutics.

Strug, Lisa J; Gonska, Tanja; He, Gengming; Keenan, Katherine; Ip, Wan; Boëlle, Pierre-Yves; Lin, Fan; Panjwani, Naim; Gong, Jiafen; Li, Weili; Soave, David; Xiao, Bowei; Tullis, Elizabeth; Rabin, Harvey; Parkins, Michael D; Price, April; Zuberbuhler, Peter C; Corvol, Harriet; Ratjen, Felix; Sun, Lei; Bear, Christine E; Rommens, Johanna M.

Hum Mol Genet ; 25(20): 4590-4600, 2016 10 15.

Artículo en Inglés | MEDLINE | ID: mdl-28171547

16.

Current insights into the role of PKA phosphorylation in CFTR channel activity and the pharmacological rescue of cystic fibrosis disease-causing mutants.

Chin, Stephanie; Hung, Maurita; Bear, Christine E.

Cell Mol Life Sci ; 74(1): 57-66, 2017 01.

Artículo en Inglés | MEDLINE | ID: mdl-27722768

17.

Channel Gating Regulation by the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) First Cytosolic Loop.

Ehrhardt, Annette; Chung, W Joon; Pyle, Louise C; Wang, Wei; Nowotarski, Krzysztof; Mulvihill, Cory M; Ramjeesingh, Mohabir; Hong, Jeong; Velu, Sadanandan E; Lewis, Hal A; Atwell, Shane; Aller, Steve; Bear, Christine E; Lukacs, Gergely L; Kirk, Kevin L; Sorscher, Eric J.

J Biol Chem ; 291(4): 1854-1865, 2016 Jan 22.

Artículo en Inglés | MEDLINE | ID: mdl-26627831

18.

The major cystic fibrosis causing mutation exhibits defective propensity for phosphorylation.

Pasyk, Stan; Molinski, Steven; Ahmadi, Saumel; Ramjeesingh, Mohabir; Huan, Ling-Jun; Chin, Stephanie; Du, Kai; Yeger, Herman; Taylor, Paul; Moran, Michael F; Bear, Christine E.

Proteomics ; 15(2-3): 447-61, 2015 Jan.

Artículo en Inglés | MEDLINE | ID: mdl-25330774

19.

The cystic fibrosis transmembrane conductance regulator is an extracellular chloride sensor.

Broadbent, Steven D; Ramjeesingh, Mohabir; Bear, Christine E; Argent, Barry E; Linsdell, Paul; Gray, Michael A.

Pflugers Arch ; 467(8): 1783-94, 2015 Aug.

Artículo en Inglés | MEDLINE | ID: mdl-25277268

20.

Genetic, cell biological, and clinical interrogation of the CFTR mutation c.3700 A>G (p.Ile1234Val) informs strategies for future medical intervention.

Molinski, Steven V; Gonska, Tanja; Huan, Ling Jun; Baskin, Berivan; Janahi, Ibrahim A; Ray, Peter N; Bear, Christine E.

Genet Med ; 16(8): 625-32, 2014 Aug.

Artículo en Inglés | MEDLINE | ID: mdl-24556927

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