Christine Bear - Publications

Affiliations: 
Biochemistry University of Toronto, Toronto, ON, Canada 
Area:
Cell Biology, Physiology Biology, Pharmacology, Biochemistry

125/222 high-probability publications. We are testing a new system for linking publications to authors. You can help! If you notice any inaccuracies, please sign in and mark papers as correct or incorrect matches. If you identify any major omissions or other inaccuracies in the publication list, please let us know.

Year Citation  Score
2023 Birimberg-Schwartz L, Ip W, Bartlett C, Avolio J, Vonk AM, Gunawardena T, Du K, Esmaeili M, Beekman JM, Rommens J, Strug L, Bear CE, Moraes TJ, Gonska T. Validating organoid-derived human intestinal monolayers for personalized therapy in cystic fibrosis. Life Science Alliance. 6. PMID 37024122 DOI: 10.26508/lsa.202201857  0.303
2022 Xia S, Di Paola M, Jones NL, Bear CE. A Fluorescence-based Assay of Membrane Potential for High-throughput Functional Study of Two Endogenous Ion Channels in Two Epithelial Cell Lines. Journal of Visualized Experiments : Jove. PMID 35815993 DOI: 10.3791/63528  0.377
2022 Lim SH, Snider J, Birimberg-Schwartz L, Ip W, Serralha JC, Botelho HM, Lopes-Pacheco M, Pinto MC, Moutaoufik MT, Zilocchi M, Laselva O, Esmaeili M, Kotlyar M, Lyakisheva A, Tang P, ... ... Bear CE, et al. CFTR interactome mapping using the mammalian membrane two-hybrid high-throughput screening system. Molecular Systems Biology. 18: e10629. PMID 35156780 DOI: 10.15252/msb.202110629  0.446
2021 Xia S, Bozóky Z, Di Paola M, Laselva O, Ahmadi S, Jiang JX, Pitstick AL, Jiang C, Rotin D, Mayhew CN, Jones NL, Bear CE. High-Throughput Functional Analysis of CFTR and Other Apically Localized Proteins in iPSC-Derived Human Intestinal Organoids. Cells. 10. PMID 34943927 DOI: 10.3390/cells10123419  0.52
2021 Derichs N, Taylor-Cousar JL, Davies JC, Fajac I, Tullis E, Nazareth D, Downey DG, Rosenbluth D, Malfroot A, Saunders C, Jensen R, Solomon GM, Vermeulen F, Kaiser A, Willmann S, ... ... Bear CE, et al. Riociguat for the treatment of Phe508del homozygous adults with cystic fibrosis. Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society. PMID 34419414 DOI: 10.1016/j.jcf.2021.07.015  0.329
2021 Laselva O, Qureshi Z, Zeng ZW, Petrotchenko EV, Ramjeesingh M, Hamilton CM, Huan LJ, Borchers CH, Pomès R, Young R, Bear CE. Identification of binding sites for ivacaftor on the cystic fibrosis transmembrane conductance regulator. Iscience. 24: 102542. PMID 34142049 DOI: 10.1016/j.isci.2021.102542  0.316
2021 Laselva O, Ardelean MC, Bear CE. Phenotyping Rare CFTR Mutations Reveal Functional Expression Defects Restored by TRIKAFTA. Journal of Personalized Medicine. 11. PMID 33920764 DOI: 10.3390/jpm11040301  0.338
2020 Laselva O, McCormack J, Bartlett C, Ip W, Gunawardena TNA, Ouyang H, Eckford PDW, Gonska T, Moraes TJ, Bear CE. Preclinical Studies of a Rare CF-Causing Mutation in the Second Nucleotide Binding Domain (c.3700A>G) Show Robust Functional Rescue in Primary Nasal Cultures by Novel CFTR Modulators. Journal of Personalized Medicine. 10. PMID 33167369 DOI: 10.3390/jpm10040209  0.363
2020 Laselva O, Bartlett C, Popa A, Ouyang H, Gunawardena TNA, Gonska T, Moraes TJ, Bear CE. Emerging preclinical modulators developed for F508del-CFTR have the potential to be effective for ORKAMBI resistant processing mutants. Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society. PMID 32741662 DOI: 10.1016/j.jcf.2020.07.015  0.31
2020 Erwood S, Laselva O, Bily TMI, Brewer RA, Rutherford AH, Bear CE, Ivakine EA. Allele-Specific Prevention of Nonsense-Mediated Decay in Cystic Fibrosis Using Homology-Independent Genome Editing. Molecular Therapy. Methods & Clinical Development. 17: 1118-1128. PMID 32490033 DOI: 10.1016/j.omtm.2020.05.002  0.328
2020 Cao H, Ouyang H, Laselva O, Bartlett C, Zhou ZP, Duan C, Gunawardena T, Avolio J, Bear CE, Gonska T, Hu J, Moraes TJ. A helper-dependent adenoviral vector rescues CFTR to wild type functional levels in CF epithelial cells harbouring class I mutations. The European Respiratory Journal. PMID 32457197 DOI: 10.1183/13993003.00205-2020  0.423
2020 Laselva O, Moraes TJ, He G, Bartlett C, Szàrics I, Ouyang H, Gunawardena TNA, Strug L, Bear CE, Gonska T. The CFTR Mutation c.3453G > C (D1152H) Confers an Anion Selectivity Defect in Primary Airway Tissue that Can Be Rescued by Ivacaftor. Journal of Personalized Medicine. 10. PMID 32414100 DOI: 10.3390/Jpm10020040  0.458
2020 Leir SH, Yin S, Kerschner JL, Xia S, Ahmadi S, Bear C, Harris A. An organoid model to assay the role of CFTR in the human epididymis epithelium. Cell and Tissue Research. PMID 32377875 DOI: 10.1007/S00441-020-03208-7  0.493
2020 Xia S, Laselva O, Bear CE, Jones N. A115 MODELING CYSTIC FIBROSIS (CF) INTESTINAL DISEASE USING PATIENT DERIVED TISSUES Journal of the Canadian Association of Gastroenterology. 3: 133-134. DOI: 10.1093/jcag/gwz047.114  0.449
2019 Laselva O, Erwood S, Du K, Ivakine Z, Bear CE. Activity of lumacaftor is not conserved in zebrafish Cftr bearing the major cystic fibrosis-causing mutation. Faseb Bioadvances. 1: 661-670. PMID 32123813 DOI: 10.1096/fba.2019-00039  0.4
2019 Laselva O, Eckford PD, Bartlett C, Ouyang H, Gunawardena TN, Gonska T, Moraes TJ, Bear CE. Functional rescue of c.3846G>A (W1282X) in patient-derived nasal cultures achieved by inhibition of nonsense mediated decay and protein modulators with complementary mechanisms of action. Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society. PMID 31831337 DOI: 10.1016/j.jcf.2019.12.001  0.385
2019 Wu YS, Jiang J, Ahmadi S, Lew A, Laselva O, Xia S, Bartlett C, Ip W, Wellhauser L, Ouyang H, Gonska T, Moraes TJ, Bear C. ORKAMBI®mediated rescue of mucociliary clearance in CF primary respiratory cultures is enhanced by arginine uptake, arginase inhibition and promotion of nitric oxide signaling to the CFTR channel. Molecular Pharmacology. PMID 31427400 DOI: 10.1124/mol.119.117143  0.433
2019 Chin S, Ramjeesingh M, Hung M, Ereño-Oreba J, Cui H, Laselva O, Julien JP, Bear CE. Cholesterol Interaction Directly Enhances Intrinsic Activity of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR). Cells. 8. PMID 31370288 DOI: 10.3390/cells8080804  0.416
2019 Ahmadi S, Wu YS, Li M, Ip W, Lloyd-Kuzik A, Di Paola M, Du K, Xia S, Lew A, Bozoky Z, Forman-Kay J, Bear CE, Gonska T. Augmentation of CFTR function in human bronchial epithelial cells via SLC6A14-dependent amino acid uptake: Implications for treatment of Cystic Fibrosis. American Journal of Respiratory Cell and Molecular Biology. PMID 31189070 DOI: 10.1165/Rcmb.2019-0094Oc  0.439
2019 Ocana SC, Wong A, Aushev M, Yang JY, Perkins N, Bear C, Rossant J, Gray M. How can gene-editing of human pluripotent stem cells help cystic fibrosis? European Respiratory Journal. DOI: 10.1183/13993003.Congress-2019.Oa2125  0.362
2018 Laselva O, Marzaro G, Vaccarin C, Lampronti I, Tamanini A, Lippi G, Gambari R, Cabrini G, Bear CE, Chilin A, Dechecchi MC. Molecular Mechanism of Action of Trimethylangelicin Derivatives as CFTR Modulators. Frontiers in Pharmacology. 9: 719. PMID 30022950 DOI: 10.3389/fphar.2018.00719  0.345
2018 Ahmadi S, Xia S, Wu YS, Di Paola M, Kissoon R, Luk C, Lin F, Du K, Rommens J, Bear C. SLC6A14, an amino acid transporter, modifies the primary CF defect in fluid secretion. Elife. 7. PMID 30004386 DOI: 10.7554/eLife.37963  0.467
2018 Chin S, Hung M, Won A, Wu YS, Ahmadi S, Yang D, Elmallah S, Toutah K, Hamilton CM, Young RN, Viirre RD, Yip CM, Bear CE. Lipophilicity of the Cystic Fibrosis drug, Ivacaftor, and its destabilizing effect on the major CF-causing mutation: F508del. Molecular Pharmacology. PMID 29903751 DOI: 10.1124/mol.118.112177  0.399
2018 Cao H, Ouyang H, Grasemann H, Bartlett C, Du K, Duan R, Shi F, Estrada M, Seigel K, Coates A, Yeger H, Bear C, Gonska T, Moraes T, Hu J. Transducing airway basal cells with a helper-dependent adenoviral vector for lung gene therapy. Human Gene Therapy. PMID 29320887 DOI: 10.1089/Hum.2017.201  0.355
2018 Hamilton CM, Hung M, Chen G, Qureshi Z, Thompson JR, Sun B, Bear CE, Young RN. Synthesis and characterization of a photoaffinity labelling probe based on the structure of the cystic fibrosis drug ivacaftor Tetrahedron. 74: 5528-5538. DOI: 10.1016/J.Tet.2018.06.016  0.355
2017 Di Paola M, Park AJ, Ahmadi S, Roach EJ, Wu YS, Struder-Kypke M, Lam JS, Bear CE, Khursigara CM. SLC6A14 Is a Genetic Modifier of Cystic Fibrosis That Regulates Pseudomonas aeruginosa Attachment to Human Bronchial Epithelial Cells. Mbio. 8. PMID 29259090 DOI: 10.1128/mBio.02073-17  0.337
2017 Guo L, Karoubi G, Duchesneau P, Shutova MV, Sung HK, Tonge P, Bear C, Rogers I, Nagy A, Waddell TK. Generation of Induced Progenitor-like Cells from Mature Epithelial Cells Using Interrupted Reprogramming. Stem Cell Reports. 9: 1780-1795. PMID 29198829 DOI: 10.1016/J.Stemcr.2017.10.022  0.367
2017 Ahmadi S, Bozoky Z, Di Paola M, Xia S, Li C, Wong AP, Wellhauser L, Molinski SV, Ip W, Ouyang H, Avolio J, Forman-Kay JD, Ratjen F, Hirota JA, Rommens J, ... ... Bear CE, et al. Phenotypic profiling of CFTR modulators in patient-derived respiratory epithelia. Npj Genomic Medicine. 2: 12. PMID 28649446 DOI: 10.1038/S41525-017-0015-6  0.304
2017 Bozoky Z, Ahmadi S, Milman T, Kim TH, Du K, Di Paola M, Pasyk S, Pekhletski R, Keller JP, Bear CE, Forman-Kay JD. Synergy of cAMP and calcium signaling pathways in CFTR regulation. Proceedings of the National Academy of Sciences of the United States of America. PMID 28242698 DOI: 10.1073/Pnas.1613546114  0.364
2016 Strug LJ, Gonska T, He G, Keenan K, Ip W, Boëlle PY, Lin F, Panjwani N, Gong J, Li W, Soave D, Xiao B, Tullis E, Rabin H, Parkins MD, ... ... Bear CE, et al. Cystic fibrosis gene modifier SLC26A9 modulates airway response to CFTR-directed therapeutics. Human Molecular Genetics. 25: 4590-4600. PMID 28171547 DOI: 10.1093/Hmg/Ddw290  0.383
2016 Chin S, Yang D, Miles AJ, Eckford PD, Molinski SV, Wallace BA, Bear CE. Attenuation of phosphorylation-dependent activation of cystic fibrosis transmembrane conductance regulator (CFTR) by disease-causing mutations at the transmission interface. The Journal of Biological Chemistry. PMID 28003367 DOI: 10.1074/jbc.M116.762633  0.359
2016 Chin S, Hung M, Bear CE. Current insights into the role of PKA phosphorylation in CFTR channel activity and the pharmacological rescue of cystic fibrosis disease-causing mutants. Cellular and Molecular Life Sciences : Cmls. PMID 27722768 DOI: 10.1007/s00018-016-2388-6  0.413
2016 Laselva O, Molinski S, Casavola V, Bear CE. The investigational Cystic Fibrosis drug Trimethylangelicin directly modulates CFTR by stabilizing the first membrane-spanning domain. Biochemical Pharmacology. PMID 27614011 DOI: 10.1016/j.bcp.2016.09.005  0.368
2016 Strug LJ, Gonska T, He G, Keenan K, Ip W, Boelle PY, Lin F, Panjwani N, Gong J, Li W, Soave D, Xiao B, Tullis E, Rabin H, Parkins MD, ... ... Bear CE, et al. Cystic Fibrosis Gene Modifier SLC26A9 Modulates Airway Response to CFTR-directed Therapeutics. Human Molecular Genetics. PMID 27571897 DOI: 10.1093/hmg/ddw290  0.383
2016 Ahmadi S, Xia S, Di Paola M, Ip W, Rommens J, Gonska T, Bear CE. SLC6A14 Enhances CFTR Channel Activity in the Cystic Fibrosis Affected Lung Epithelium Biophysical Journal. 110: 452a. DOI: 10.1016/j.bpj.2015.11.2427  0.393
2015 Cao H, Ouyang H, Ip W, Du K, Duan W, Avolio J, Wu J, Duan C, Yeger H, Bear CE, Gonska T, Hu J, Moraes TJ. Testing gene therapy vectors in human primary nasal epithelial cultures. Molecular Therapy. Methods & Clinical Development. 2: 15034. PMID 26730394 DOI: 10.1038/Mtm.2015.34  0.432
2015 Ehrhardt A, Chung WJ, Pyle LC, Wang W, Nowotarski K, Mulvihill CM, Ramjeesingh M, Hong J, Velu SE, Lewis HA, Atwell S, Aller S, Bear CE, Lukacs GL, Kirk KL, et al. Channel Gating Regulation by the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) First Cytosolic Loop. The Journal of Biological Chemistry. PMID 26627831 DOI: 10.1074/Jbc.M115.704809  0.476
2015 Verkman AS, Edelman A, Amaral M, Mall MA, Beekman JM, Meiners T, Galietta LJ, Bear CE. Finding new drugs to enhance anion secretion in cystic fibrosis: Toward suitable systems for better drug screening. Report on the pre-conference meeting to the 12th ECFS Basic Science Conference, Albufeira, 25-28 March 2015. Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society. 14: 700-5. PMID 26474804 DOI: 10.1016/J.Jcf.2015.10.001  0.355
2015 Molinski SV, Ahmadi S, Hung M, Bear CE. Facilitating Structure-Function Studies of CFTR Modulator Sites with Efficiencies in Mutagenesis and Functional Screening. Journal of Biomolecular Screening. 20: 1204-17. PMID 26385858 DOI: 10.1177/1087057115605834  0.321
2015 Ogawa M, Ogawa S, Bear CE, Ahmadi S, Chin S, Li B, Grompe M, Keller G, Kamath BM, Ghanekar A. Directed differentiation of cholangiocytes from human pluripotent stem cells. Nature Biotechnology. 33: 853-61. PMID 26167630 DOI: 10.1038/Nbt.3294  0.361
2015 Eckford PD, Li C, Bear CE. Functional reconstitution and channel activity measurements of purified wildtype and mutant CFTR protein. Journal of Visualized Experiments : Jove. PMID 25867140 DOI: 10.3791/52427  0.45
2015 Pasyk S, Molinski S, Ahmadi S, Ramjeesingh M, Huan LJ, Chin S, Du K, Yeger H, Taylor P, Moran MF, Bear CE. The major cystic fibrosis causing mutation exhibits defective propensity for phosphorylation. Proteomics. 15: 447-61. PMID 25330774 DOI: 10.1002/Pmic.201400218  0.409
2015 Broadbent SD, Ramjeesingh M, Bear CE, Argent BE, Linsdell P, Gray MA. The cystic fibrosis transmembrane conductance regulator is an extracellular chloride sensor. PflüGers Archiv : European Journal of Physiology. 467: 1783-94. PMID 25277268 DOI: 10.1007/s00424-014-1618-8  0.412
2015 Chin S, Ramjeesingh M, Eckford P, Bear C. Investigating the Effect of PKA Phosphorylation on Intramolecular Interactions in Purified Full Length Wildtype CFTR Biophysical Journal. 108: 382a-383a. DOI: 10.1016/J.Bpj.2014.11.2097  0.409
2015 Ahmadi S, Luk C, Xia S, Di Paola M, Chung T, Rommens J, Bear C. SLC6A14 Modifies Fluid Secretory Capacity of Cystic Fibrosis Affected Epithelium by Enhancing CFTR Channel Function Biophysical Journal. 108: 19a-20a. DOI: 10.1016/J.Bpj.2014.11.132  0.61
2014 Jaecklin T, Duerr J, Huang H, Rafii M, Bear CE, Ratjen F, Pencharz P, Kavanagh BP, Mall MA, Grasemann H. Lung arginase expression and activity is increased in cystic fibrosis mouse models. Journal of Applied Physiology (Bethesda, Md. : 1985). 117: 284-8. PMID 24925982 DOI: 10.1152/Japplphysiol.00167.2014  0.374
2014 Eckford PD, Ramjeesingh M, Molinski S, Pasyk S, Dekkers JF, Li C, Ahmadi S, Ip W, Chung TE, Du K, Yeger H, Beekman J, Gonska T, Bear CE. VX-809 and related corrector compounds exhibit secondary activity stabilizing active F508del-CFTR after its partial rescue to the cell surface. Chemistry & Biology. 21: 666-78. PMID 24726831 DOI: 10.1016/J.Chembiol.2014.02.021  0.327
2013 Ahmadi S, Luk C, Chung T, Mangat R, Rommens J, Bear C. Role of SLC6A14 as a Modifier of Cystic Fibrosis Phenotype Biophysical Journal. 104: 22a. DOI: 10.1016/J.Bpj.2012.11.160  0.572
2012 Molinski S, Eckford PD, Pasyk S, Ahmadi S, Chin S, Bear CE. Functional Rescue of F508del-CFTR Using Small Molecule Correctors. Frontiers in Pharmacology. 3: 160. PMID 23055971 DOI: 10.3389/fphar.2012.00160  0.388
2012 Eckford PD, Li C, Ramjeesingh M, Bear CE. Cystic fibrosis transmembrane conductance regulator (CFTR) potentiator VX-770 (ivacaftor) opens the defective channel gate of mutant CFTR in a phosphorylation-dependent but ATP-independent manner. The Journal of Biological Chemistry. 287: 36639-49. PMID 22942289 DOI: 10.1074/jbc.M112.393637  0.486
2012 Wong AP, Bear CE, Chin S, Pasceri P, Thompson TO, Huan LJ, Ratjen F, Ellis J, Rossant J. Directed differentiation of human pluripotent stem cells into mature airway epithelia expressing functional CFTR protein. Nature Biotechnology. 30: 876-82. PMID 22922672 DOI: 10.1038/Nbt.2328  0.435
2011 Alkhouri B, Denning RA, Kim Chiaw P, Eckford PD, Yu W, Li C, Bogojeski JJ, Bear CE, Viirre RD. Synthesis and properties of molecular probes for the rescue site on mutant cystic fibrosis transmembrane conductance regulator. Journal of Medicinal Chemistry. 54: 8693-701. PMID 22074181 DOI: 10.1021/jm201335c  0.365
2011 Kim Chiaw P, Eckford PD, Bear CE. Insights into the mechanisms underlying CFTR channel activity, the molecular basis for cystic fibrosis and strategies for therapy. Essays in Biochemistry. 50: 233-48. PMID 21967060 DOI: 10.1042/bse0500233  0.493
2011 Eckford PD, Bear CE. Targeting the regulation of CFTR channels. The Biochemical Journal. 435: e1-4. PMID 21726198 DOI: 10.1042/BJ20110461  0.366
2011 Yu W, Kim Chiaw P, Bear CE. Probing conformational rescue induced by a chemical corrector of F508del-cystic fibrosis transmembrane conductance regulator (CFTR) mutant. The Journal of Biological Chemistry. 286: 24714-25. PMID 21602569 DOI: 10.1074/jbc.M111.239699  0.445
2011 Malik FA, Meissner A, Bear C, Bolz SS. Sphingosine-1-Phosphate acutely modulates the Cystic Fibrosis Transmembrane Conductance Regulator Protein in an S1P2 and AMPK-dependent manner The Faseb Journal. 25. DOI: 10.1096/Fasebj.25.1_Supplement.746.6  0.33
2010 Kim Chiaw P, Wellhauser L, Huan LJ, Ramjeesingh M, Bear CE. A chemical corrector modifies the channel function of F508del-CFTR. Molecular Pharmacology. 78: 411-8. PMID 20501743 DOI: 10.1124/mol.110.065862  0.449
2010 Wellhauser L, D'Antonio C, Bear CE. ClC transporters: discoveries and challenges in defining the mechanisms underlying function and regulation of ClC-5. PflüGers Archiv : European Journal of Physiology. 460: 543-57. PMID 20049483 DOI: 10.1007/s00424-009-0769-5  0.35
2009 Kim Chiaw P, Huan LJ, Gagnon S, Ly D, Sweezey N, Rotin D, Deber CM, Bear CE. Functional rescue of DeltaF508-CFTR by peptides designed to mimic sorting motifs. Chemistry & Biology. 16: 520-30. PMID 19477416 DOI: 10.1016/J.Chembiol.2009.04.005  0.363
2009 Wellhauser L, Kim Chiaw P, Pasyk S, Li C, Ramjeesingh M, Bear CE. A small-molecule modulator interacts directly with deltaPhe508-CFTR to modify its ATPase activity and conformational stability. Molecular Pharmacology. 75: 1430-8. PMID 19339490 DOI: 10.1124/mol.109.055608  0.441
2009 Pasyk S, Li C, Ramjeesingh M, Bear CE. Direct interaction of a small-molecule modulator with G551D-CFTR, a cystic fibrosis-causing mutation associated with severe disease. The Biochemical Journal. 418: 185-90. PMID 18945216 DOI: 10.1042/BJ20081424  0.462
2008 Cheung JC, Kim Chiaw P, Pasyk S, Bear CE. Molecular basis for the ATPase activity of CFTR. Archives of Biochemistry and Biophysics. 476: 95-100. PMID 18417076 DOI: 10.1016/j.abb.2008.03.033  0.371
2008 Payandeh J, Li C, Ramjeesingh M, Poduch E, Bear CE, Pai EF. Probing structure-function relationships and gating mechanisms in the CorA Mg2+ transport system. The Journal of Biological Chemistry. 283: 11721-33. PMID 18276588 DOI: 10.1074/Jbc.M707889200  0.301
2008 Ramjeesingh M, Ugwu F, Stratford FL, Huan LJ, Li C, Bear CE. The intact CFTR protein mediates ATPase rather than adenylate kinase activity. The Biochemical Journal. 412: 315-21. PMID 18241200 DOI: 10.1042/BJ20071719  0.378
2008 Dhani SU, Kim Chiaw P, Huan LJ, Bear CE. ATP depletion inhibits the endocytosis of ClC-2. Journal of Cellular Physiology. 214: 273-80. PMID 17620322 DOI: 10.1002/jcp.21192  0.407
2008 Gonska T, Ip W, Bear C, Durie P, Quintan P. β-adrenergic stimulation alters sweat gland potential difference in cystic fibrosis (CF) patients but not healthy controls Journal of Cystic Fibrosis. 7: S24. DOI: 10.1016/S1569-1993(08)60091-0  0.375
2006 Ramjeesingh M, Li C, She YM, Bear CE. Evaluation of the membrane-spanning domain of ClC-2. The Biochemical Journal. 396: 449-60. PMID 16526942 DOI: 10.1042/BJ20060043  0.323
2006 Dhani SU, Bear CE. Role of intramolecular and intermolecular interactions in ClC channel and transporter function. PflüGers Archiv : European Journal of Physiology. 451: 708-15. PMID 16167151 DOI: 10.1007/s00424-005-1513-4  0.358
2004 Kogan I, Ramjeesingh M, Bear CE. ATPase assay of purified, reconstituted CFTR protein. Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society. 3: 133-4. PMID 15463945 DOI: 10.1016/J.Jcf.2004.05.028  0.66
2004 Norez C, Heda GD, Jensen T, Kogan I, Hughes LK, Auzanneau C, Dérand R, Bulteau-Pignoux L, Li C, Ramjeesingh M, Li H, Sheppard DN, Bear CE, Riordan JR, Becq F. Determination of CFTR chloride channel activity and pharmacology using radiotracer flux methods. Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society. 3: 119-21. PMID 15463942 DOI: 10.1016/J.Jcf.2004.05.025  0.671
2004 Sheppard DN, Gray MA, Gong X, Sohma Y, Kogan I, Benos DJ, Scott-Ward TS, Chen JH, Li H, Cai Z, Gupta J, Li C, Ramjeesingh M, Berdiev BK, Ismailov II, ... Bear CE, et al. The patch-clamp and planar lipid bilayer techniques: powerful and versatile tools to investigate the CFTR Cl- channel. Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society. 3: 101-8. PMID 15463939 DOI: 10.1016/J.Jcf.2004.05.046  0.617
2004 Benos DJ, Berdiev BK, Ismailov II, Ostedgaard LS, Kogan I, Li C, Ramjeesingh M, Bear CE. Methods to study CFTR protein in vitro. Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society. 3: 79-83. PMID 15463933 DOI: 10.1016/J.Jcf.2004.05.018  0.667
2004 Kidd JF, Kogan I, Bear CE. Molecular basis for the chloride channel activity of cystic fibrosis transmembrane conductance regulator and the consequences of disease-causing mutations. Current Topics in Developmental Biology. 60: 215-49. PMID 15094300 DOI: 10.1016/S0070-2153(04)60007-X  0.718
2003 Ramjeesingh M, Ugwu F, Li C, Dhani S, Huan LJ, Wang Y, Bear CE. Stable dimeric assembly of the second membrane-spanning domain of CFTR (cystic fibrosis transmembrane conductance regulator) reconstitutes a chloride-selective pore. The Biochemical Journal. 375: 633-41. PMID 12892562 DOI: 10.1042/BJ20030774  0.385
2003 Ramjeesingh M, Kidd JF, Huan LJ, Wang Y, Bear CE. Dimeric cystic fibrosis transmembrane conductance regulator exists in the plasma membrane. The Biochemical Journal. 374: 793-7. PMID 12820897 DOI: 10.1042/BJ20030683  0.404
2003 Mohammad-Panah R, Harrison R, Dhani S, Ackerley C, Huan LJ, Wang Y, Bear CE. The chloride channel ClC-4 contributes to endosomal acidification and trafficking. The Journal of Biological Chemistry. 278: 29267-77. PMID 12746443 DOI: 10.1074/Jbc.M304357200  0.417
2003 Kogan I, Ramjeesingh M, Li C, Kidd JF, Wang Y, Leslie EM, Cole SP, Bear CE. CFTR directly mediates nucleotide-regulated glutathione flux. The Embo Journal. 22: 1981-9. PMID 12727866 DOI: 10.1093/Emboj/Cdg194  0.656
2003 Dhani SU, Mohammad-Panah R, Ahmed N, Ackerley C, Ramjeesingh M, Bear CE. Evidence for a functional interaction between the ClC-2 chloride channel and the retrograde motor dynein complex. The Journal of Biological Chemistry. 278: 16262-70. PMID 12601004 DOI: 10.1074/jbc.M209828200  0.325
2002 Kidd JF, Bear CE. Epithelial cell chloride channel activity correlates with improved airway function in cystic fibrosis patients with the major mutant: Delta F508. Pediatric Research. 52: 625-7. PMID 12409505 DOI: 10.1203/00006450-200211000-00004  0.395
2002 Pan J, Bear C, Farragher S, Cutz E, Yeger H. Cystic fibrosis transmembrane conductance regulator modulates neurosecretory function in pulmonary neuroendocrine cell-related tumor cell line models. American Journal of Respiratory Cell and Molecular Biology. 27: 553-60. PMID 12397014 DOI: 10.1165/Rcmb.4843  0.417
2002 Kogan I, Ramjeesingh M, Li C, Bear CE. Studies of the molecular basis for cystic fibrosis using purified reconstituted CFTR protein. Methods in Molecular Medicine. 70: 143-57. PMID 11917519 DOI: 10.1385/1-59259-187-6:143  0.595
2002 Mohammad-Panah R, Ackerley C, Rommens J, Choudhury M, Wang Y, Bear CE. The chloride channel ClC-4 co-localizes with cystic fibrosis transmembrane conductance regulator and may mediate chloride flux across the apical membrane of intestinal epithelia. The Journal of Biological Chemistry. 277: 566-74. PMID 11675385 DOI: 10.1074/jbc.M106968200  0.568
2001 Gyömörey K, Garami E, Galley K, Rommens JM, Bear CE. Non-CFTR chloride channels likely contribute to secretion in the murine small intestine. PflüGers Archiv : European Journal of Physiology. 443: S103-6. PMID 11845313 DOI: 10.1007/S004240100654  0.787
2001 Ramjeesingh M, Li C, Kogan I, Wang Y, Huan LJ, Bear CE. A monomer is the minimum functional unit required for channel and ATPase activity of the cystic fibrosis transmembrane conductance regulator. Biochemistry. 40: 10700-6. PMID 11524016 DOI: 10.1021/Bi0108195  0.701
2001 Yeger H, Pan J, Fu XW, Bear C, Cutz E. Expression of CFTR and Cl- conductances in cells of pulmonary neuroepithelial bodies American Journal of Physiology - Lung Cellular and Molecular Physiology. 281. PMID 11504700 DOI: 10.1152/Ajplung.2001.281.3.L713  0.459
2001 Kogan I, Ramjeesingh M, Huan LJ, Wang Y, Bear CE. Perturbation of the pore of the cystic fibrosis transmembrane conductance regulator (CFTR) inhibits its atpase activity. The Journal of Biological Chemistry. 276: 11575-81. PMID 11124965 DOI: 10.1074/Jbc.M010403200  0.636
2001 Mohammad-Panah R, Gyomorey K, Rommens J, Choudhury M, Li C, Wang Y, Bear CE. ClC-2 contributes to native chloride secretion by a human intestinal cell line, Caco-2. The Journal of Biological Chemistry. 276: 8306-13. PMID 11096079 DOI: 10.1074/Jbc.M006764200  0.774
2000 Ahmed N, Ramjeesingh M, Wong S, Varga A, Garami E, Bear CE. Chloride channel activity of CIC-2 is modified by the actin cytoskeleton Biochemical Journal. 352: 789-794. PMID 11104687 DOI: 10.1042/0264-6021:3520789  0.314
2000 Gyömörey K, Rozmahel R, Bear CE. Amelioration of intestinal disease severity in cystic fibrosis mice is associated with improved chloride secretory capacity. Pediatric Research. 48: 731-4. PMID 11102538 DOI: 10.1203/00006450-200012000-00005  0.766
2000 Gyömörey K, Yeger H, Ackerley C, Garami E, Bear CE. Expression of the chloride channel ClC-2 in the murine small intestine epithelium. American Journal of Physiology. Cell Physiology. 279: C1787-94. PMID 11078693 DOI: 10.1152/Ajpcell.2000.279.6.C1787  0.775
2000 Ramjeesingh M, Li C, Huan LJ, Garami E, Wang Y, Bear CE. Quaternary structure of the chloride channel ClC-2 Biochemistry. 39: 13838-13847. PMID 11076524 DOI: 10.1021/bi001282i  0.424
1999 Ramjeesingh M, Li C, Garami E, Huan LJ, Galley K, Wang Y, Bear CE. Walker mutations reveal loose relationship between catalytic and channel-gating activities of purified CFTR (cystic fibrosis transmembrane conductance regulator) Biochemistry. 38: 1463-1468. PMID 9931011 DOI: 10.1021/bi982243y  0.423
1999 Xiong H, Li C, Garami E, Wang Y, Ramjeesingh M, Galley K, Bear CE. ClC-2 activation modulates regulatory volume decrease Journal of Membrane Biology. 167: 215-221. PMID 9929373 DOI: 10.1007/s002329900485  0.339
1999 Pasyk EA, Morin XK, Zeman P, Garami E, Galley K, Huan LJ, Wang Y, Bear CE. A conserved region of the R domain of cystic fibrosis transmembrane conductance regulator is important in processing and function Journal of Biological Chemistry. 273: 31759-31764. PMID 9822639 DOI: 10.1074/jbc.273.48.31759  0.327
1998 Ramjeesingh M, Garami E, Galley K, Li C, Wang Y, Bear CE. Purification and reconstitution of epithelial chloride channel cystic fibrosis transmembrane conductance regulator Methods in Enzymology. 294: 227-246. PMID 9916230 DOI: 10.1016/S0076-6879(99)94014-4  0.497
1998 Li C, Breton S, Morrison R, Cannon CL, Emma F, Sanchez-Olea R, Bear C, Strange K. Recombinant pICln forms highly cation-selective channels when reconstituted into artificial and biological membranes. The Journal of General Physiology. 112: 727-36. PMID 9834142 DOI: 10.1085/Jgp.112.6.727  0.376
1998 Ramjeesingh M, Huan LJ, Wilschanski M, Durie P, Li C, Gyomorey K, Wang Y, Kent G, Tanswell KA, Cutz E, Ackerley C, Bear CE. Assessment of the efficacy of in vivo CFTR protein replacement therapy in CF mice. Human Gene Therapy. 9: 521-8. PMID 9525313 DOI: 10.1089/Hum.1998.9.4-521  0.77
1998 Rozmahel R, Nguyen V, Corey M, Haston CK, Kent G, Bear C, Durie P, Tsui LC. Defining the modifiers of CF pathophysiology Pediatric Pulmonology. 26: 126.  0.319
1997 Bear CE, Li C, Galley K, Wang Y, Garami E, Ramjeesingh M. Coupling of ATP hydrolysis with channel gating by purified, reconstituted CFTR Journal of Bioenergetics and Biomembranes. 29: 465-473. PMID 9511931 DOI: 10.1023/A:1022435007193  0.435
1997 Kent G, Iles R, Bear CE, Huan LJ, Griesenbach U, McKerlie C, Frndova H, Ackerley C, Gosselin D, Radzioch D, O'Brodovich H, Tsui LC, Buchwald M, Tanswell AK. Lung disease in mice with cystic fibrosis. The Journal of Clinical Investigation. 100: 3060-9. PMID 9399953 DOI: 10.1172/Jci119861  0.344
1997 Ramjeesingh M, Li C, Garami E, Huan LJ, Hewryk M, Wang Y, Galley K, Bear CE. A novel procedure for the efficient purification of the cystic fibrosis transmembrane conductance regulator (CFTR) Biochemical Journal. 327: 17-21. PMID 9355728  0.397
1997 Rozmahel R, Gyömörey K, Plyte S, Nguyen V, Wilschanski M, Durie P, Bear CE, Tsui LC. Incomplete rescue of cystic fibrosis transmembrane conductance regulator deficient mice by the human CFTR cDNA. Human Molecular Genetics. 6: 1153-62. PMID 9215687 DOI: 10.1093/Hmg/6.7.1153  0.779
1997 Xiong H, Garami E, Wang Y, Galley K, Ramjeesingh M, Bear C. Functional expression of CIC-2 chloride channel in Sf9 insect cells Faseb Journal. 11.  0.404
1996 Li C, Ramjeesingh M, Wang W, Garami E, Hewryk M, Lee D, Rommens JM, Galley K, Bear CE. ATPase activity of the cystic fibrosis transmembrane conductance regulator Journal of Biological Chemistry. 271: 28463-28468. PMID 8910473 DOI: 10.1074/jbc.271.45.28463  0.497
1996 Li C, Ramjeesingh M, Bear CE. Purified cystic fibrosis transmembrane conductance regulator (CFTR) does not function as an ATP channel Journal of Biological Chemistry. 271: 11623-11626. PMID 8662751 DOI: 10.1074/jbc.271.20.11623  0.474
1996 Wilschanski MA, Rozmahel R, Beharry S, Kent G, Li C, Tsui LC, Durie P, Bear CE. In vivo measurements of ion transport in long-living CF mice Biochemical and Biophysical Research Communications. 219: 753-759. PMID 8645253 DOI: 10.1006/bbrc.1996.0306  0.5
1996 Rozmahel R, Wilschanski M, Matin A, Plyte S, Oliver M, Auerbach W, Moore A, Forstner J, Durie P, Nadeau J, Bear C, Tsui LC. Modulation of disease severity in cystic fibrosis transmembrane conductance regulator deficient mice by a secondary genetic factor Nature Genetics. 12: 280-287. PMID 8589719 DOI: 10.1038/Ng0396-280  0.538
1995 O'Riordan CR, Erickson A, Bear C, Li C, Manavalan P, Wang KX, Marshall J, Scheule RK, McPherson JM, Cheng SH. Purification and characterization of recombinant cystic fibrosis transmembrane conductance regulator from Chinese hamster ovary and insect cells. The Journal of Biological Chemistry. 270: 17033-43. PMID 7542655 DOI: 10.1074/Jbc.270.28.17033  0.362
1995 Krolczyk AJ, Bear CE, Lai PFH, Schimmer BP. Effects of mutations in cAMP‐dependent protein kinase on chloride efflux in Caco‐2 human colonic carcinoma cells Journal of Cellular Physiology. 162: 64-73. PMID 7529238 DOI: 10.1002/Jcp.1041620109  0.408
1995 Morin XK, Bond TD, Loo TW, Clarke DM, Bear CE. Failure of P‐glycoprotein (MDR1) expressed in Xenopus oocytes to produce swelling‐activated chloride channel activity The Journal of Physiology. 486: 707-714. PMID 7473231 DOI: 10.1113/jphysiol.1995.sp020846  0.382
1994 Bear CE. Drugs transported by P-glycoprotein inhibit a 40pS outwardly rectifying chloride channel Biochemical and Biophysical Research Communications. 200: 513-521. PMID 7909435 DOI: 10.1006/bbrc.1994.1478  0.355
1993 Lukacs GL, Chang XB, Bear C, Kartner N, Mohamed A, Riordan JR, Grinstein S. The ΔF508 mutation decreases the stability of cystic fibrosis transmembrane conductance regulator in the plasma membrane: Determination of functional half-lives on transfected cells Journal of Biological Chemistry. 268: 21592-21598. PMID 7691813  0.342
1993 O'Brodovich H, Wang X, Li C, Rafii B, Correa J, Bear C. Novobiocin forms cation-permeable ion channels in rat fetal distal lung epithelium. The American Journal of Physiology. 264: C1532-7. PMID 7687410 DOI: 10.1152/Ajpcell.1993.264.6.C1532  0.342
1993 Bear C, Ling V. Multidrug resistance and cystic fibrosis genes: complementarity of function? Trends in Genetics : Tig. 9: 67-8. PMID 7683829 DOI: 10.1016/0168-9525(93)90214-3  0.324
1993 Li C, Ramjeesingh M, Reyes E, Jensen T, Chang X, Rommens JM, Bear CE. The cystic fibrosis mutation (ΔF508) does not influence the chloride channel activity of CFTR Nature Genetics. 3: 311-316. PMID 7526932 DOI: 10.1038/ng0493-311  0.492
1992 Sood R, Bear C, Auerbach W, Reyes E, Jensen T, Kartner N, Riordan JR, Buchwald M. Regulation of CFTR expression function during differentiation of intestinal epithelial cells Embo Journal. 11: 2487-2494. PMID 1378393  0.336
1992 Bear CE, Li C, Kartner N, Bridges RJ, Jensen TJ, Ramjeesingh M, Riordan JR. Purification and functional reconstitution of the cystic fibrosis transmembrane conductance regulator (CFTR) Cell. 68: 809-818. PMID 1371239 DOI: 10.1016/0092-8674(92)90155-6  0.481
1992 Bear CE, Reyes EF. cAMP-activated chloride conductance in the colonic cell line, Caco-2 American Journal of Physiology - Cell Physiology. 262. PMID 1370744  0.361
1992 Sood R, Bear C, Auerbach W, Reyes E, Jensen T, Kartner N, Riordan JR, Buchwald M. Regulation of CFTR expression and function during differentiation of intestinal epithelial cells. The Embo Journal. 11: 2487-2494. DOI: 10.1002/J.1460-2075.1992.Tb05313.X  0.442
1991 Bear CE. A K+-selective channel in the colonic carcinoma cell line: CaCo-2 is activated with membrane stretch Bba - Biomembranes. 1069: 267-272. PMID 1932066 DOI: 10.1016/0005-2736(91)90134-T  0.36
1991 Bear CE, Duguay F, Naismith AL, Kartner N, Hanrahan JW, Riordan JR. Cl- channel activity in Xenopus oocytes expressing the cystic fibrosis gene Journal of Biological Chemistry. 266: 19142-19145. PMID 1717461  0.43
1991 Rommens JM, Dho S, Bear CE, Kartner N, Kennedy D, Riordan JR, Tsui LC, Foskett JK. cAMP-inducible chloride conductance in mouse fibroblast lines stably expressing the human cystic fibrosis transmembrane conductance regulator Proceedings of the National Academy of Sciences of the United States of America. 88: 7500-7504. PMID 1715567  0.485
1991 Kartner N, Hanrahan JW, Jensen TJ, Naismith AL, Sun S, Ackerley CA, Reyes EF, Tsui LC, Rommens JM, Bear CE, Riordan JR. Expression of the cystic fibrosis gene in non-epithelial invertebrate cells produces a regulated anion conductance Cell. 64: 681-691. PMID 1705179 DOI: 10.1016/0092-8674(91)90498-N  0.479
1988 Bear CE. Phosphorylation-activated chloride channels in human skin fibroblasts Febs Letters. 237: 145-149. PMID 2458964 DOI: 10.1016/0014-5793(88)80189-3  0.433
1985 Bear CE, Petrunka CN, Strasberg SM. Evidence for a channel for the electrogenic transport of chloride ion in the rat hepatocyte. Hepatology (Baltimore, Md.). 5: 383-91. PMID 2581880 DOI: 10.1002/hep.1840050307  0.323
Low-probability matches (unlikely to be authored by this person)
2015 Wong AP, Chin S, Xia S, Garner J, Bear CE, Rossant J. Efficient generation of functional CFTR-expressing airway epithelial cells from human pluripotent stem cells. Nature Protocols. 10: 363-81. PMID 25654755 DOI: 10.1038/Nprot.2015.021  0.299
2015 Malik FA, Meissner A, Semenkov I, Molinski S, Pasyk S, Ahmadi S, Bui HH, Bear CE, Lidington D, Bolz SS. Sphingosine-1-Phosphate Is a Novel Regulator of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Activity. Plos One. 10: e0130313. PMID 26079370 DOI: 10.1371/Journal.Pone.0130313  0.297
2021 Ogawa M, Jiang JX, Xia S, Yang D, Ding A, Laselva O, Hernandez M, Cui C, Higuchi Y, Suemizu H, Dorrell C, Grompe M, Bear CE, Ogawa S. Generation of functional ciliated cholangiocytes from human pluripotent stem cells. Nature Communications. 12: 6504. PMID 34764255 DOI: 10.1038/s41467-021-26764-0  0.297
2021 Dumas MP, Xia S, Bear CE, Ratjen F. Perspectives on the translation of in-vitro studies to precision medicine in Cystic Fibrosis. Ebiomedicine. 73: 103660. PMID 34740114 DOI: 10.1016/j.ebiom.2021.103660  0.291
2004 Kidd JF, Ramjeesingh M, Stratford F, Huan LJ, Bear CE. A heteromeric complex of the two nucleotide binding domains of cystic fibrosis transmembrane conductance regulator (CFTR) mediates ATPase activity. The Journal of Biological Chemistry. 279: 41664-9. PMID 15284228 DOI: 10.1074/jbc.M407666200  0.29
1987 Bear CE, Petersen OH. L-alanine evokes opening of single Ca2+-activated K+ channels in rat liver cells PflüGers Archiv European Journal of Physiology. 410: 342-344. PMID 2446259 DOI: 10.1007/BF00580288  0.284
2012 Du K, Karp P, Zabner J, Bear C, Cutz E, Yeger H. WS2.2 Partially degraded N-terminal half delF508 CFTR molecule produces aggregation rearrangement of intermediate filaments in fully differentiated human bronchial epithelium Journal of Cystic Fibrosis. 11: S3. DOI: 10.1016/S1569-1993(12)60011-3  0.282
1991 Bear CE, Li C. Calcium-permeable channels in rat hepatoma cells are activated by extracellular nucleotides American Journal of Physiology - Cell Physiology. 261. PMID 1662901  0.281
2023 Sahakyan Y, Abrahamyan L, Ratjen F, Bear C, Strug L, Eckford PDW, Peel JK, Krahn M, Sander B. Cost-effectiveness analysis of genetic tools to predict treatment response in patients with cystic fibrosis. Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society. PMID 37100704 DOI: 10.1016/j.jcf.2023.04.013  0.28
2018 Chang YH, Stone TA, Chin S, Glibowicka M, Bear CE, Deber CM. Structural effects of extracellular loop mutations in CFTR helical hairpins. Biochimica Et Biophysica Acta. PMID 29307731 DOI: 10.1016/j.bbamem.2018.01.003  0.276
2013 D'Antonio C, Molinski S, Ahmadi S, Huan LJ, Wellhauser L, Bear CE. Conformational defects underlie proteasomal degradation of Dent's disease-causing mutants of ClC-5. The Biochemical Journal. 452: 391-400. PMID 23566014 DOI: 10.1042/BJ20121848  0.272
2013 Islam ST, Eckford PD, Jones ML, Nugent T, Bear CE, Vogel C, Lam JS. Proton-dependent gating and proton uptake by Wzx support O-antigen-subunit antiport across the bacterial inner membrane. Mbio. 4: e00678-13. PMID 24023388 DOI: 10.1128/mBio.00678-13  0.271
2018 Molinski SV, Shahani VM, Subramanian AS, MacKinnon SS, Woollard G, Laforet M, Laselva O, Morayniss LD, Bear CE, Windemuth A. Comprehensive mapping of Cystic Fibrosis mutations to CFTR protein identifies mutation clusters and molecular docking predicts corrector binding site. Proteins. PMID 29569753 DOI: 10.1002/prot.25496  0.27
2011 Whitney JC, Hay ID, Li C, Eckford PD, Robinson H, Amaya MF, Wood LF, Ohman DE, Bear CE, Rehm BH, Howell PL. Structural basis for alginate secretion across the bacterial outer membrane. Proceedings of the National Academy of Sciences of the United States of America. 108: 13083-8. PMID 21778407 DOI: 10.1073/Pnas.1104984108  0.269
2009 Mohammad-Panah R, Wellhauser L, Steinberg BE, Wang Y, Huan LJ, Liu XD, Bear CE. An essential role for ClC-4 in transferrin receptor function revealed in studies of fibroblasts derived from Clcn4-null mice. Journal of Cell Science. 122: 1229-37. PMID 19339555 DOI: 10.1242/jcs.037317  0.269
1990 Bear CE. A nonselective cation channel in rat liver cells is activated by membrane stretch American Journal of Physiology - Cell Physiology. 258. PMID 1690515  0.266
2020 Laselva O, Stone TA, Bear CE, Deber CM. Anti-Infectives Restore ORKAMBI Rescue of F508del-CFTR Function in Human Bronchial Epithelial Cells Infected with Clinical Strains of . Biomolecules. 10. PMID 32092967 DOI: 10.3390/biom10020334  0.266
2017 Molinski SV, Ahmadi S, Ip W, Ouyang H, Villella A, Miller JP, Lee PS, Kulleperuma K, Du K, Di Paola M, Eckford PD, Laselva O, Huan LJ, Wellhauser L, Li E, ... ... Bear CE, et al. Orkambi® and amplifier co-therapy improves function from a rare CFTR mutation in gene-edited cells and patient tissue. Embo Molecular Medicine. PMID 28667089 DOI: 10.15252/Emmm.201607137  0.266
2023 Cuevas-Ocaña S, Yang JY, Aushev M, Schlossmacher G, Bear CE, Hannan NRF, Perkins ND, Rossant J, Wong AP, Gray MA. A Cell-Based Optimised Approach for Rapid and Efficient Gene Editing of Human Pluripotent Stem Cells. International Journal of Molecular Sciences. 24. PMID 37373413 DOI: 10.3390/ijms241210266  0.265
1988 Bear CE, Shaffer EA. Hepatocellular water and electrolyte solution Canadian Journal of Physiology and Pharmacology. 66: 1253-1260. PMID 3071394 DOI: 10.1139/y88-206  0.265
2022 Laselva O, Petrotchenko EV, Hamilton CM, Qureshi Z, Borchers CH, Young RN, Bear CE. A protocol for identifying the binding sites of small molecules on the cystic fibrosis transmembrane conductance regulator (CFTR) protein. Star Protocols. 3: 101258. PMID 35434660 DOI: 10.1016/j.xpro.2022.101258  0.261
2020 Laselva O, Bartlett C, Gunawardena TNA, Ouyang H, Eckford PDW, Moraes TJ, Bear CE, Gonska T. 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. The European Respiratory Journal. PMID 33303536 DOI: 10.1183/13993003.02774-2020  0.256
2022 Ngan SY, Quach HT, Laselva O, Huang EN, Mangos M, Xia S, Bear CE, Wong AP. Stage-Specific Generation of Human Pluripotent Stem Cell Derived Lung Models to Measure CFTR Function. Current Protocols. 2: e341. PMID 35025140 DOI: 10.1002/cpz1.341  0.255
2018 Eckford PDW, McCormack J, Munsie L, He G, Stanojevic S, Pereira SL, Ho K, Avolio J, Bartlett C, Yang JY, Wong AP, Wellhauser L, Huan LJ, Jiang JX, Ouyang H, ... ... Bear CE, et al. The CF Canada-Sick Kids Program in individual CF therapy: A resource for the advancement of personalized medicine in CF. Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society. PMID 29685812 DOI: 10.1016/J.Jcf.2018.03.013  0.252
2019 Cao H, Ouyang H, Laselva O, Bartlett C, Bear C, Gonska T, Moraes T, Hu J. WS13-6 Adenoviral vector gene therapy results in a wild type CFTR functional pattern in class I mutation cystic fibrosis cells Journal of Cystic Fibrosis. 18: S25. DOI: 10.1016/S1569-1993(19)30197-3  0.252
2011 Wellhauser L, Luna-Chavez C, D'Antonio C, Tainer J, Bear CE. ATP induces conformational changes in the carboxyl-terminal region of ClC-5. The Journal of Biological Chemistry. 286: 6733-41. PMID 21173145 DOI: 10.1074/Jbc.M110.175877  0.241
1987 Bear CE, Davison JS, Shaffer EA. Sodium-dependent taurocholate uptake by isolated rat hepatocytes occurs through an electrogenic mechanism. Biochimica Et Biophysica Acta. 903: 388-94. PMID 2443174 DOI: 10.1016/0005-2736(87)90230-6  0.237
2007 Stratford FL, Ramjeesingh M, Cheung JC, Huan LJ, Bear CE. The Walker B motif of the second nucleotide-binding domain (NBD2) of CFTR plays a key role in ATPase activity by the NBD1-NBD2 heterodimer. The Biochemical Journal. 401: 581-6. PMID 16989640 DOI: 10.1042/BJ20060968  0.234
2014 Molinski SV, Gonska T, Huan LJ, Baskin B, Janahi IA, Ray PN, Bear CE. Genetic, cell biological, and clinical interrogation of the CFTR mutation c.3700 A>G (p.Ile1234Val) informs strategies for future medical intervention. Genetics in Medicine : Official Journal of the American College of Medical Genetics. 16: 625-32. PMID 24556927 DOI: 10.1038/gim.2014.4  0.232
2021 Oren YS, Avizur-Barchad O, Ozeri-Galai E, Elgrabli R, Schirelman MR, Blinder T, Stampfer CD, Ordan M, Laselva O, Cohen-Cymberknoh M, Kerem E, Bear CE, Kerem B. Antisense oligonucleotide splicing modulation as a novel Cystic Fibrosis therapeutic approach for the W1282X nonsense mutation. Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society. PMID 34972649 DOI: 10.1016/j.jcf.2021.12.012  0.231
2019 Wong AP, Shojaie S, Liang Q, Xia S, Di Paola M, Ahmadi S, Bilodeau C, Garner J, Post M, Duchesneau P, Waddell TK, Bear CE, Nagy A, Rossant J. Conversion of human and mouse fibroblasts into lung-like epithelial cells. Scientific Reports. 9: 9027. PMID 31227724 DOI: 10.1038/S41598-019-45195-Y  0.231
2021 Jiang JX, Wellhauser L, Laselva O, Utkina I, Bozoky Z, Gunawardena T, Ngan Z, Xia S, Di Paola M, Eckford PDW, Ratjen F, Moraes TJ, Parkinson J, Wong AP, Bear CE. A new platform for high-throughput therapy testing on iPSC-derived lung progenitor cells from cystic fibrosis patients. Stem Cell Reports. PMID 34678210 DOI: 10.1016/j.stemcr.2021.09.020  0.228
2018 Laselva O, Molinski S, Casavola V, Bear CE. Correctors of the major Cystic Fibrosis mutant interact through membrane spanning domains. Molecular Pharmacology. PMID 29618585 DOI: 10.1124/mol.118.111799  0.22
2006 Wellhauser L, Kuo HH, Stratford FL, Ramjeesingh M, Huan LJ, Luong W, Li C, Deber CM, Bear CE. Nucleotides bind to the C-terminus of ClC-5. The Biochemical Journal. 398: 289-94. PMID 16686597 DOI: 10.1042/BJ20060142  0.212
2023 Gunawardena TNA, Bozóky Z, Bartlett C, Ouyang H, Eckford PDW, Moraes TJ, Ratjen F, Gonska T, Bear CE. Correlation of Electrophysiological and Fluorescence-Based Measurements of Modulator Efficacy in Nasal Epithelial Cultures Derived from People with Cystic Fibrosis. Cells. 12. PMID 37190083 DOI: 10.3390/cells12081174  0.212
1988 Bear CE, Davison JS, Shaffer EA. Intracellular pH influences the resting membrane potential of isolated rat hepatocytes. Biochimica Et Biophysica Acta. 944: 113-20. PMID 3179283 DOI: 10.1016/0005-2736(88)90424-5  0.212
2013 Bear CE. 50 years ago in the Journal of Pediatrics: the effect of N-acetylcysteine on the viscosity of tracheobronchial secretions in cystic fibrosis of the pancreas. The Journal of Pediatrics. 162: 85. PMID 23260312 DOI: 10.1016/j.jpeds.2012.08.006  0.208
1986 Strasberg SM, Ilson RG, Bear CE. Bile salt dependent bile flow in the rabbit: Evidence for the importance of an amiloride inhibitable pathway Canadian Journal of Physiology and Pharmacology. 64: 1316-1320. PMID 3801986  0.206
2012 Pasyk S, Molinski S, Yu W, Eckford PD, Bear CE. Identification and validation of hits from high throughput screens for CFTR modulators. Current Pharmaceutical Design. 18: 628-41. PMID 22229556 DOI: 10.2174/138161212799315957  0.203
2020 Bear CE. A Therapy for Most with Cystic Fibrosis. Cell. 180: 211. PMID 31978337 DOI: 10.1016/j.cell.2019.12.032  0.198
2010 Cortez MA, Li C, Whitehead SN, Dhani SU, D'Antonio C, Huan LJ, Bennett SA, Snead OC, Bear CE. Disruption of ClC-2 expression is associated with progressive neurodegeneration in aging mice. Neuroscience. 167: 154-62. PMID 20116415 DOI: 10.1016/j.neuroscience.2010.01.042  0.198
2024 Gao W, Kanagarajah KR, Graham E, Soon K, Veres T, Moraes TJ, Bear CE, Veldhuizen RA, Wong AP, Günther A. Collagen Tubular Airway-on-Chip for Extended Epithelial Culture and Investigation of Ventilation Dynamics. Small (Weinheim An Der Bergstrasse, Germany). e2309270. PMID 38431940 DOI: 10.1002/smll.202309270  0.197
2020 Mohamed AB, Bear C, Bear M, Korobenko A. Performance analysis of two vertical-axis hydrokinetic turbines using variational multiscale method Computers & Fluids. 200: 104432. DOI: 10.1016/J.Compfluid.2020.104432  0.196
2004 Grimard V, Li C, Ramjeesingh M, Bear CE, Goormaghtigh E, Ruysschaert JM. Phosphorylation-induced conformational changes of cystic fibrosis transmembrane conductance regulator monitored by attenuated total reflection-Fourier transform IR spectroscopy and fluorescence spectroscopy. The Journal of Biological Chemistry. 279: 5528-36. PMID 14660584 DOI: 10.1074/jbc.M311014200  0.193
2015 Shojaie S, Ermini L, Ackerley C, Wang J, Chin S, Yeganeh B, Bilodeau M, Sambi M, Rogers I, Rossant J, Bear CE, Post M. Acellular lung scaffolds direct differentiation of endoderm to functional airway epithelial cells: requirement of matrix-bound HS proteoglycans. Stem Cell Reports. 4: 419-30. PMID 25660407 DOI: 10.1016/J.Stemcr.2015.01.004  0.193
1984 Bear CE, Strasberg SM. Techniques for studying biliary secretion: electrolytes in bile. Hepatology (Baltimore, Md.). 4: 25S-30S. PMID 6090297 DOI: 10.1002/hep.1840040805  0.192
1986 Bear CE, Petrunka CN, Strasberg SM. Effect of sodium taurocholate on amiloride-inhibitable sodium uptake and on intracellular pH of isolated rat hepatocytes Journal of Laboratory and Clinical Medicine. 108: 109-116. PMID 3734584  0.19
2010 Lamhonwah AM, Bear CE, Huan LJ, Kim Chiaw P, Ackerley CA, Tein I. Cystic fibrosis transmembrane conductance regulator in human muscle: Dysfunction causes abnormal metabolic recovery in exercise. Annals of Neurology. 67: 802-8. PMID 20517942 DOI: 10.1002/ana.21982  0.183
2023 Sadras I, Kerem E, Livnat G, Sarouk I, Breuer O, Reiter J, Gileles-Hillel A, Inbar O, Cohen M, Gamliel A, Stanleigh N, Gunawardena T, Bartlett C, Gonska T, Moraes T, ... ... Bear CE, et al. Clinical and functional efficacy of elexacaftor/tezacaftor/ivacaftor in people with cystic fibrosis carrying the N1303K mutation. Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society. PMID 37331863 DOI: 10.1016/j.jcf.2023.06.001  0.179
1989 ROTHSTEIN A, BEAR C. Cell Volume Changes and the Activity of the Chloride Conductance Path Annals of the New York Academy of Sciences. 574: 294-308. PMID 2561323 DOI: 10.1111/j.1749-6632.1989.tb25166.x  0.174
2016 Chin S, Ramjeesingh M, Eckford P, Bear CE. Phosphorylation Modifies Coupling of the Membrane Domains and NBD1 of Full Length CFTR Biophysical Journal. 110: 546a. DOI: 10.1016/j.bpj.2015.11.2920  0.174
2017 Sheppard DN, Bear CE, de Jonge HR. Editorial overview: Respiratory: Transformational therapies for cystic fibrosis. Current Opinion in Pharmacology. 34: viii-xi. PMID 29221574 DOI: 10.1016/j.coph.2017.11.006  0.167
2009 Wellhauser L, Penmatsa H, Naren AP, Bear CE. Identification Of The NHERF2 Binding Site For The Chloride/Proton Transporter ClC-5 Biophysical Journal. 96: 445a-446a. DOI: 10.1016/J.Bpj.2008.12.2289  0.165
2008 Mumbengegwi DR, Li Q, Li C, Bear CE, Engelhardt JF. Evidence for a superoxide permeability pathway in endosomal membranes. Molecular and Cellular Biology. 28: 3700-12. PMID 18378695 DOI: 10.1128/Mcb.02038-07  0.164
2021 Gao W, Vaezzadeh N, Chow K, Chen H, Lavender P, Jeronimo MD, McAllister A, Laselva O, Jiang JX, Gage BK, Ogawa S, Ramchandran A, Bear CE, Keller GM, Günther A. One-Step Formation of Protein-Based Tubular Structures for Functional Devices and Tissues. Advanced Healthcare Materials. e2001746. PMID 33694327 DOI: 10.1002/adhm.202001746  0.161
1999 Ramjeesingh M, Huan LJ, Garami E, Bear CE. Novel method for evaluation of the oligomeric structure of membrane proteins Biochemical Journal. 342: 119-123. PMID 10432308 DOI: 10.1042/0264-6021:3420119  0.158
2018 Beaudoin T, Stone TA, Glibowicka M, Adams C, Yau Y, Ahmadi S, Bear CE, Grasemann H, Waters V, Deber CM. Activity of a novel antimicrobial peptide against Pseudomonas aeruginosa biofilms. Scientific Reports. 8: 14728. PMID 30283025 DOI: 10.1038/s41598-018-33016-7  0.158
2022 Rizwan M, Ling C, Guo C, Liu T, Jiang JX, Bear CE, Ogawa S, Shoichet MS. Viscoelastic Notch Signaling Hydrogel Induces Liver Bile Duct Organoid Growth and Morphogenesis. Advanced Healthcare Materials. e2200880. PMID 36180392 DOI: 10.1002/adhm.202200880  0.144
1991 Bear CE. Regulation of ion conductance in human skin fibroblasts Advances in Experimental Medicine and Biology. 290: 273-286. PMID 1719764  0.14
2011 Bear C. Being Angelica? Exploring individual animal geographies Area. 43: 297-304. DOI: 10.1111/j.1475-4762.2011.01019.x  0.129
2024 Tanjala AC, Jiang JX, Eckford PDW, Ramjeesingh M, Li C, Huan LJ, Langeveld G, Townsend C, Paone DV, Busch-Petersen J, Pekhletski R, Tang L, Raju V, Rowe SM, Bear CE. Comparison of a novel potentiator of CFTR channel activity to ivacaftor in ameliorating mucostasis caused by cigarette smoke in primary human bronchial airway epithelial cells. Biorxiv : the Preprint Server For Biology. PMID 38496440 DOI: 10.1101/2024.03.01.582742  0.12
2023 Bear C, Ratjen F. Charting the path to expanded access for CFTR modulator drugs: the nose knows. The European Respiratory Journal. 62. PMID 37857432 DOI: 10.1183/13993003.01387-2023  0.118
2009 Cheung JC, Kim Chiaw P, Deber CM, Bear CE. A novel method for monitoring the cytosolic delivery of peptide cargo. Journal of Controlled Release : Official Journal of the Controlled Release Society. 137: 2-7. PMID 19285529 DOI: 10.1016/j.jconrel.2009.02.022  0.104
1993 Lynch JJ, Wallace AA, Van der Gaag LH, Baskin EP, Bear CM, Gehret JR, Kothstein T, Stupienski RF, Appleby SD, Sanguinetti MC, Jurkiewicz NK, Zingaro GJ, Stein RB, Claremon DA, Elliott JM, et al. Cardiac electrophysiologic and antiarrhythmic actions of 3,4-dihydro-1'- [2-(benzofurazan-5-yl) ethyl]-6-methanesulfonamidospiro[(2H)-1-benzopyran- 2,4'-piperidin]-4-one HCl (L-691,121), a novel class III agent Journal of Pharmacology and Experimental Therapeutics. 265: 720-730. PMID 8496818  0.094
2014 Molinski S, Gonska T, Huan L, Baskin B, Janahi I, Ray P, Bear C. WS23.2 Characterization of the CFTR mutation c.3700A>G informs strategies for future medical intervention Journal of Cystic Fibrosis. 13: S43. DOI: 10.1016/S1569-1993(14)60133-8  0.082
2014 Bear C. Governance of the seas: A more-than-human perspective on the Cardigan Bay scallop fishery Water Worlds: Human Geographies of the Ocean. 147-162.  0.078
1987 Minuk GY, Bear CE, Sarjeant EJ. Sodium-independent, bicuculline-sensitive [3H]GABA binding to isolated rat hepatocytes American Journal of Physiology - Gastrointestinal and Liver Physiology. 252. PMID 3578523  0.078
2011 Bear C, Eden S. Thinking like a fish? Engaging with nonhuman difference through recreational angling Environment and Planning D: Society and Space. 29: 336-352. DOI: 10.1068/d1810  0.075
2013 Bear C. Assembling the sea: Materiality, movement and regulatory practices in the cardigan bay scallop fishery Cultural Geographies. 20: 21-41. DOI: 10.1177/1474474012463665  0.071
2014 Rogers A, Bear C, Hunt M, Mills S, Sandover R. Intervention: The impact agenda and human geography in UK higher education Acme. 13: 1-9.  0.062
2015 Bear C, Holloway L. Country Life: Agricultural Technologies and the Emergence of New Rural Subjectivities Geography Compass. 9: 303-315. DOI: 10.1111/gec3.12217  0.059
1986 Petersen OH, Bear C. Physiology: Two glucagon transducing systems Nature. 323: 18. PMID 3018585 DOI: 10.1038/323018a0  0.058
2008 Bear C, Eden S. Making space for fish: The regional, network and fluid spaces of fisheries certification Social and Cultural Geography. 9: 487-504. DOI: 10.1080/14649360802224358  0.055
2011 Eden S, Bear C. Reading the river through 'watercraft': Environmental engagement through knowledge and practice in freshwater angling Cultural Geographies. 18: 297-314. DOI: 10.1177/1474474010384913  0.052
1987 Bear C, Shaffer EA, Davison JS. The electrogenic effect of sodium taurocholate on rat hepatocyte couplets. Proceedings of the Western Pharmacology Society. 30: 109-11. PMID 3628259  0.051
2006 Bear C. Salmon by numbers: Quantification and understandings of nature Scottish Geographical Journal. 122: 185-203. DOI: 10.1080/00369220601100042  0.047
2001 Bear C, Shori D. Editorial to conference proceedings Pflugers Archiv European Journal of Physiology. 443. DOI: 10.1007/s004240100634  0.046
2008 Eden S, Bear C, Walker G. Understanding and (dis)trusting food assurance schemes: Consumer confidence and the 'knowledge fix' Journal of Rural Studies. 24: 1-14. DOI: 10.1016/j.jrurstud.2007.06.001  0.042
2010 Wellhauser L, Bear CE. Nucleotide Binding Induces Conformational Changes in Full-Length ClC-5 Biophysical Journal. 98: 233a. DOI: 10.1016/j.bpj.2009.12.1263  0.041
2000 Millsap BA, Bear C. Density and reproduction of burrowing owls along an urban development gradient Journal of Wildlife Management. 64: 33-41.  0.039
2012 Eden S, Bear C. The good, the bad, and the hands-on: Constructs of public participation, anglers, and lay management of water environments Environment and Planning A. 44: 1200-1240. DOI: 10.1068/a4495  0.037
2011 Eden S, Bear C. Models of equilibrium, natural agency and environmental change: Lay ecologies in UK recreational angling Transactions of the Institute of British Geographers. 36: 393-407. DOI: 10.1111/j.1475-5661.2011.00438.x  0.037
2008 Eden S, Bear C, Walker G. Mucky carrots and other proxies: Problematising the knowledge-fix for sustainable and ethical consumption Geoforum. 39: 1044-1057. DOI: 10.1016/j.geoforum.2007.11.001  0.025
2010 Eden S, Bear C. Third-sector global environmental governance, space and science: Comparing fishery and forestry certification Journal of Environmental Policy and Planning. 12: 83-106. DOI: 10.1080/15239081003626000  0.024
2013 Shrinivas L, Bodagala S, Varadarajan R, Cary A, Bharathan V, Bear C. Materialization strategies in the Vertica analytic database: Lessons learned Proceedings - International Conference On Data Engineering. 1196-1207. DOI: 10.1109/ICDE.2013.6544909  0.024
1989 Bear CL, Willsteed EM, Zagarella SS. Lichen nitidus confined to one palm Australasian Journal of Dermatology. 30: 41-42. PMID 2486051  0.023
2014 Holloway L, Bear C, Wilkinson K. Robotic milking technologies and renegotiating situated ethical relationships on UK dairy farms Agriculture and Human Values. 31: 185-199. DOI: 10.1007/s10460-013-9473-3  0.023
2000 Seradge H, Bear C, Bithell D. Preventing carpal tunnel syndrome and cumulative trauma disorder: effect of carpal tunnel decompression excercises: an Oklahoma experience The Journal of the Oklahoma State Medical Association. 93: 150-153. PMID 10793505  0.022
2000 Bear CL, Montgomery WA, Nolte DB, Rajchel SK, Silva MC. Open, programmable networks Bell Labs Technical Journal. 5: 30-42. DOI: 10.1002/bltj.2234  0.021
2014 Holloway L, Bear C, Wilkinson K. Re-capturing bovine life: Robot-cow relationships, freedom and control in dairy farming Journal of Rural Studies. 33: 131-140. DOI: 10.1016/j.jrurstud.2013.01.006  0.019
2011 Holloway L, Bear C. DNA typing and super dairies: Changing practices and remaking cows reenacting dairy cows Environment and Planning A. 43: 1487-1491. DOI: 10.1068/a4425  0.019
2008 Eden S, Bear C, Walker G. The sceptical consumer? Exploring views about food assurance Food Policy. 33: 624-630. DOI: 10.1016/j.foodpol.2008.02.003  0.018
2007 Stonebraker M, Bear C, Çetintemel U, Cherniack M, Ge T, Hachem N, Harizopoulos S, Lifter J, Rogers J, Zdonik S. One size fits all? - Part 2: Benchmarking results Cidr 2007 - 3rd Biennial Conference On Innovative Data Systems Research. 173-184.  0.017
2012 Bear C, Lamb A, Tran N. The vertica database: SQL RDBMS for managing big data Mbds'12 - Proceedings of the 2012 Workshop On Management of Big Data Systems, Co-Located With Icac'12. 37-38. DOI: 10.1145/2378356.2378367  0.01
2012 Lamb A, Fuller M, Varadarajan R, Tran N, Vandiver B, Doshi L, Bear C. The vertica analytic database: Cstore 7 years later Proceedings of the Vldb Endowment. 5: 1790-1801.  0.01
2011 Bear C, Bull J. Guest editorial Environment and Planning A. 43: 2261-2266. DOI: 10.1068/a44498  0.01
1992 Bear CC, Gibson SW. Applications using dry gas seals to control axial shaft loads in centrifugal compressors Asme 1992 International Gas Turbine and Aeroengine Congress and Exposition, Gt 1992. 3. DOI: 10.1115/92-GT-104  0.01
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