| 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 |
|
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