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Molecular Mechanism of Action of Trimethylangelicin Derivatives as CFTR Modulators.

Laselva O, Marzaro G, Vaccarin C, Lampronti I, Tamanini A, Lippi G, Gambari R, Cabrini G, Bear CE, Chilin A, Dechecchi MC.

Front Pharmacol. 2018 Jul 4;9:719. doi: 10.3389/fphar.2018.00719. eCollection 2018.


SLC6A14, an amino acid transporter, modifies the primary CF defect in fluid secretion.

Ahmadi S, Xia S, Wu YS, Di Paola M, Kissoon R, Luk C, Lin F, Du K, Rommens J, Bear CE.

Elife. 2018 Jul 13;7. pii: e37963. doi: 10.7554/eLife.37963.


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

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.

Mol Pharmacol. 2018 Aug;94(2):917-925. doi: 10.1124/mol.118.112177. Epub 2018 Jun 14.


The CF Canada-Sick Kids Program in individual CF therapy: A resource for the advancement of personalized medicine in CF.

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, Du K, Klingel M, Kyriakopoulou L, Gonska T, Moraes TJ, Strug LJ, Rossant J, Ratjen F, Bear CE.

J Cyst Fibros. 2018 Apr 20. pii: S1569-1993(18)30086-9. doi: 10.1016/j.jcf.2018.03.013. [Epub ahead of print]


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

Laselva O, Molinski S, Casavola V, Bear CE.

Mol Pharmacol. 2018 Jun;93(6):612-618. doi: 10.1124/mol.118.111799. Epub 2018 Apr 4.


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

Molinski SV, Shahani VM, Subramanian AS, MacKinnon SS, Woollard G, Laforet M, Laselva O, Morayniss LD, Bear CE, Windemuth A.

Proteins. 2018 Aug;86(8):833-843. doi: 10.1002/prot.25496. Epub 2018 Apr 10.


Transducing Airway Basal Cells with a Helper-Dependent Adenoviral Vector for Lung Gene Therapy.

Cao H, Ouyang H, Grasemann H, Bartlett C, Du K, Duan R, Shi F, Estrada M, Seigel KE, Coates AL, Yeger H, Bear CE, Gonska T, Moraes TJ, Hu J.

Hum Gene Ther. 2018 Jun;29(6):643-652. doi: 10.1089/hum.2017.201. Epub 2018 Mar 16.


Structural effects of extracellular loop mutations in CFTR helical hairpins.

Chang YH, Stone TA, Chin S, Glibowicka M, Bear CE, Deber CM.

Biochim Biophys Acta Biomembr. 2018 May;1860(5):1092-1098. doi: 10.1016/j.bbamem.2018.01.003. Epub 2018 Jan 5.


SLC6A14 Is a Genetic Modifier of Cystic Fibrosis That Regulates Pseudomonas aeruginosa Attachment to Human Bronchial Epithelial Cells.

Di Paola M, Park AJ, Ahmadi S, Roach EJ, Wu YS, Struder-Kypke M, Lam JS, Bear CE, Khursigara CM.

MBio. 2017 Dec 19;8(6). pii: e02073-17. doi: 10.1128/mBio.02073-17.


Editorial overview: Respiratory: Transformational therapies for cystic fibrosis.

Sheppard DN, Bear CE, de Jonge HR.

Curr Opin Pharmacol. 2017 Jun;34:viii-xi. doi: 10.1016/j.coph.2017.11.006. No abstract available.


Orkambi® and amplifier co-therapy improves function from a rare CFTR mutation in gene-edited cells and patient tissue.

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, Ray PN, Pomès R, Moraes TJ, Gonska T, Ratjen F, Bear CE.

EMBO Mol Med. 2017 Sep;9(9):1224-1243. doi: 10.15252/emmm.201607137.


Phenotypic profiling of CFTR modulators in patient-derived respiratory epithelia.

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, Rossant J, Gonska T, Moraes TJ, Bear CE.

NPJ Genom Med. 2017 Apr 14;2:12. doi: 10.1038/s41525-017-0015-6.


Synergy of cAMP and calcium signaling pathways in CFTR regulation.

Bozoky Z, Ahmadi S, Milman T, Kim TH, Du K, Di Paola M, Pasyk S, Pekhletski R, Keller JP, Bear CE, Forman-Kay JD.

Proc Natl Acad Sci U S A. 2017 Mar 14;114(11):E2086-E2095. doi: 10.1073/pnas.1613546114. Epub 2017 Feb 27.


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

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, Price A, Zuberbuhler PC, Corvol H, Ratjen F, Sun L, Bear CE, Rommens JM.

Hum Mol Genet. 2016 Oct 15;25(20):4590-4600. doi: 10.1093/hmg/ddw290.


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

Chin S, Yang D, Miles AJ, Eckford PD, Molinski S, Wallace BA, Bear CE.

J Biol Chem. 2017 Feb 3;292(5):1988-1999. doi: 10.1074/jbc.M116.762633. Epub 2016 Dec 21.


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

Chin S, Hung M, Bear CE.

Cell Mol Life Sci. 2017 Jan;74(1):57-66. doi: 10.1007/s00018-016-2388-6. Epub 2016 Oct 8. Review.


The investigational Cystic Fibrosis drug Trimethylangelicin directly modulates CFTR by stabilizing the first membrane-spanning domain.

Laselva O, Molinski S, Casavola V, Bear CE.

Biochem Pharmacol. 2016 Nov 1;119:85-92. doi: 10.1016/j.bcp.2016.09.005. Epub 2016 Sep 8.


Testing gene therapy vectors in human primary nasal epithelial cultures.

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.

Mol Ther Methods Clin Dev. 2015 Sep 9;2:15034. doi: 10.1038/mtm.2015.34. eCollection 2015.


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

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, Sorscher EJ.

J Biol Chem. 2016 Jan 22;291(4):1854-65. doi: 10.1074/jbc.M115.704809. Epub 2015 Dec 1.


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.

Verkman AS, Edelman A, Amaral M, Mall MA, Beekman JM, Meiners T, Galietta LJ, Bear CE.

J Cyst Fibros. 2015 Nov;14(6):700-5. doi: 10.1016/j.jcf.2015.10.001. Epub 2015 Oct 21. No abstract available.


Facilitating Structure-Function Studies of CFTR Modulator Sites with Efficiencies in Mutagenesis and Functional Screening.

Molinski SV, Ahmadi S, Hung M, Bear CE.

J Biomol Screen. 2015 Dec;20(10):1204-17. doi: 10.1177/1087057115605834. Epub 2015 Sep 18.


Directed differentiation of cholangiocytes from human pluripotent stem cells.

Ogawa M, Ogawa S, Bear CE, Ahmadi S, Chin S, Li B, Grompe M, Keller G, Kamath BM, Ghanekar A.

Nat Biotechnol. 2015 Aug;33(8):853-61. doi: 10.1038/nbt.3294. Epub 2015 Jul 13.


Sphingosine-1-Phosphate Is a Novel Regulator of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Activity.

Malik FA, Meissner A, Semenkov I, Molinski S, Pasyk S, Ahmadi S, Bui HH, Bear CE, Lidington D, Bolz SS.

PLoS One. 2015 Jun 16;10(6):e0130313. doi: 10.1371/journal.pone.0130313. eCollection 2015.


Acellular lung scaffolds direct differentiation of endoderm to functional airway epithelial cells: requirement of matrix-bound HS proteoglycans.

Shojaie S, Ermini L, Ackerley C, Wang J, Chin S, Yeganeh B, Bilodeau M, Sambi M, Rogers I, Rossant J, Bear CE, Post M.

Stem Cell Reports. 2015 Mar 10;4(3):419-30. doi: 10.1016/j.stemcr.2015.01.004. Epub 2015 Feb 5.


Efficient generation of functional CFTR-expressing airway epithelial cells from human pluripotent stem cells.

Wong AP, Chin S, Xia S, Garner J, Bear CE, Rossant J.

Nat Protoc. 2015 Mar;10(3):363-81. doi: 10.1038/nprot.2015.021. Epub 2015 Feb 5.


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

Pasyk S, Molinski S, Ahmadi S, Ramjeesingh M, Huan LJ, Chin S, Du K, Yeger H, Taylor P, Moran MF, Bear CE.

Proteomics. 2015 Jan;15(2-3):447-61. doi: 10.1002/pmic.201400218. Epub 2014 Dec 17.


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

Broadbent SD, Ramjeesingh M, Bear CE, Argent BE, Linsdell P, Gray MA.

Pflugers Arch. 2015 Aug;467(8):1783-94. doi: 10.1007/s00424-014-1618-8. Epub 2014 Oct 4.


Lung arginase expression and activity is increased in cystic fibrosis mouse models.

Jaecklin T, Duerr J, Huang H, Rafii M, Bear CE, Ratjen F, Pencharz P, Kavanagh BP, Mall MA, Grasemann H.

J Appl Physiol (1985). 2014 Aug 1;117(3):284-8. doi: 10.1152/japplphysiol.00167.2014. Epub 2014 Jun 12.


VX-809 and related corrector compounds exhibit secondary activity stabilizing active F508del-CFTR after its partial rescue to the cell surface.

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.

Chem Biol. 2014 May 22;21(5):666-78. doi: 10.1016/j.chembiol.2014.02.021. Epub 2014 Apr 10.


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

Molinski SV, Gonska T, Huan LJ, Baskin B, Janahi IA, Ray PN, Bear CE.

Genet Med. 2014 Aug;16(8):625-32. doi: 10.1038/gim.2014.4. Epub 2014 Feb 20.


Proton-dependent gating and proton uptake by Wzx support O-antigen-subunit antiport across the bacterial inner membrane.

Islam ST, Eckford PD, Jones ML, Nugent T, Bear CE, Vogel C, Lam JS.

MBio. 2013 Sep 10;4(5):e00678-13. doi: 10.1128/mBio.00678-13.


Conformational defects underlie proteasomal degradation of Dent's disease-causing mutants of ClC-5.

D'Antonio C, Molinski S, Ahmadi S, Huan LJ, Wellhauser L, Bear CE.

Biochem J. 2013 Jun 15;452(3):391-400. doi: 10.1042/BJ20121848.


Functional Rescue of F508del-CFTR Using Small Molecule Correctors.

Molinski S, Eckford PD, Pasyk S, Ahmadi S, Chin S, Bear CE.

Front Pharmacol. 2012 Sep 26;3:160. doi: 10.3389/fphar.2012.00160. eCollection 2012.


Directed differentiation of human pluripotent stem cells into mature airway epithelia expressing functional CFTR protein.

Wong AP, Bear CE, Chin S, Pasceri P, Thompson TO, Huan LJ, Ratjen F, Ellis J, Rossant J.

Nat Biotechnol. 2012 Sep;30(9):876-82.


Identification and validation of hits from high throughput screens for CFTR modulators.

Pasyk S, Molinski S, Yu W, Eckford PD, Bear CE.

Curr Pharm Des. 2012;18(5):628-41. Review.


Synthesis and properties of molecular probes for the rescue site on mutant cystic fibrosis transmembrane conductance regulator.

Alkhouri B, Denning RA, Kim Chiaw P, Eckford PD, Yu W, Li C, Bogojeski JJ, Bear CE, Viirre RD.

J Med Chem. 2011 Dec 22;54(24):8693-701. doi: 10.1021/jm201335c. Epub 2011 Nov 21.


Insights into the mechanisms underlying CFTR channel activity, the molecular basis for cystic fibrosis and strategies for therapy.

Kim Chiaw P, Eckford PD, Bear CE.

Essays Biochem. 2011 Sep 7;50(1):233-48. doi: 10.1042/bse0500233. Review.


Structural basis for alginate secretion across the bacterial outer membrane.

Whitney JC, Hay ID, Li C, Eckford PD, Robinson H, Amaya MF, Wood LF, Ohman DE, Bear CE, Rehm BH, Howell PL.

Proc Natl Acad Sci U S A. 2011 Aug 9;108(32):13083-8. doi: 10.1073/pnas.1104984108. Epub 2011 Jul 21.


Targeting the regulation of CFTR channels.

Eckford PD, Bear CE.

Biochem J. 2011 Apr 15;435(2):e1-4. doi: 10.1042/BJ20110461.


Probing conformational rescue induced by a chemical corrector of F508del-cystic fibrosis transmembrane conductance regulator (CFTR) mutant.

Yu W, Kim Chiaw P, Bear CE.

J Biol Chem. 2011 Jul 15;286(28):24714-25. doi: 10.1074/jbc.M111.239699. Epub 2011 May 21.


ATP induces conformational changes in the carboxyl-terminal region of ClC-5.

Wellhauser L, Luna-Chavez C, D'Antonio C, Tainer J, Bear CE.

J Biol Chem. 2011 Feb 25;286(8):6733-41. doi: 10.1074/jbc.M110.175877. Epub 2010 Dec 20.


Cystic fibrosis transmembrane conductance regulator in human muscle: Dysfunction causes abnormal metabolic recovery in exercise.

Lamhonwah AM, Bear CE, Huan LJ, Kim Chiaw P, Ackerley CA, Tein I.

Ann Neurol. 2010 Jun;67(6):802-8. doi: 10.1002/ana.21982.


A chemical corrector modifies the channel function of F508del-CFTR.

Kim Chiaw P, Wellhauser L, Huan LJ, Ramjeesingh M, Bear CE.

Mol Pharmacol. 2010 Sep;78(3):411-8. doi: 10.1124/mol.110.065862. Epub 2010 May 25.


Disruption of ClC-2 expression is associated with progressive neurodegeneration in aging mice.

Cortez MA, Li C, Whitehead SN, Dhani SU, D'Antonio C, Huan LJ, Bennett SA, Snead OC 3rd, Bear CE.

Neuroscience. 2010 Apr 28;167(1):154-62. doi: 10.1016/j.neuroscience.2010.01.042. Epub 2010 Jan 29.


ClC transporters: discoveries and challenges in defining the mechanisms underlying function and regulation of ClC-5.

Wellhauser L, D'Antonio C, Bear CE.

Pflugers Arch. 2010 Jul;460(2):543-57. doi: 10.1007/s00424-009-0769-5. Epub 2010 Jan 5. Review.


Functional rescue of DeltaF508-CFTR by peptides designed to mimic sorting motifs.

Kim Chiaw P, Huan LJ, Gagnon S, Ly D, Sweezey N, Rotin D, Deber CM, Bear CE.

Chem Biol. 2009 May 29;16(5):520-30. doi: 10.1016/j.chembiol.2009.04.005.


An essential role for ClC-4 in transferrin receptor function revealed in studies of fibroblasts derived from Clcn4-null mice.

Mohammad-Panah R, Wellhauser L, Steinberg BE, Wang Y, Huan LJ, Liu XD, Bear CE.

J Cell Sci. 2009 Apr 15;122(Pt 8):1229-37. doi: 10.1242/jcs.037317.

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