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Hsp70 and DNAJA2 limit CFTR levels through degradation.

Kim Chiaw P, Hantouche C, Wong MJH, Matthes E, Robert R, Hanrahan JW, Shrier A, Young JC.

PLoS One. 2019 Aug 13;14(8):e0220984. doi: 10.1371/journal.pone.0220984. eCollection 2019.


Magnetic microboats for floating, stiffness tunable, air-liquid interface epithelial cultures.

Chandrasekaran A, Kouthouridis S, Lee W, Lin N, Ma Z, Turner MJ, Hanrahan JW, Moraes C.

Lab Chip. 2019 Sep 7;19(17):2786-2798. doi: 10.1039/c9lc00267g. Epub 2019 Jul 23.


Bioactive Thymosin Alpha-1 Does Not Influence F508del-CFTR Maturation and Activity.

Armirotti A, Tomati V, Matthes E, Veit G, Cholon DM, Phuan PW, Braccia C, Guidone D, Gentzsch M, Lukacs GL, Verkman AS, Galietta LJV, Hanrahan JW, Pedemonte N.

Sci Rep. 2019 Jul 16;9(1):10310. doi: 10.1038/s41598-019-46639-1.


Cystic Fibrosis: Proteostatic correctors of CFTR trafficking and alternative therapeutic targets.

Hanrahan JW, Sato Y, Carlile GW, Jansen G, Young JC, Thomas DY.

Expert Opin Ther Targets. 2019 Aug;23(8):711-724. doi: 10.1080/14728222.2019.1628948. Epub 2019 Jun 9.


Agonists that stimulate secretion promote the recruitment of CFTR into membrane lipid microdomains.

Abu-Arish A, Pandžić E, Kim D, Tseng HW, Wiseman PW, Hanrahan JW.

J Gen Physiol. 2019 Jun 3;151(6):834-849. doi: 10.1085/jgp.201812143. Epub 2019 May 2.


Pendrin Mediates Bicarbonate Secretion and Enhances Cystic Fibrosis Transmembrane Conductance Regulator Function in Airway Surface Epithelia.

Kim D, Huang J, Billet A, Abu-Arish A, Goepp J, Matthes E, Tewfik MA, Frenkiel S, Hanrahan JW.

Am J Respir Cell Mol Biol. 2019 Jun;60(6):705-716. doi: 10.1165/rcmb.2018-0158OC.


Variable Responses to CFTR Correctors in vitro: Estimating the Design Effect in Precision Medicine.

Matthes E, Goepp J, Martini C, Shan J, Liao J, Thomas DY, Hanrahan JW.

Front Pharmacol. 2018 Dec 19;9:1490. doi: 10.3389/fphar.2018.01490. eCollection 2018.


A novel triple combination of pharmacological chaperones improves F508del-CFTR correction.

Carlile GW, Yang Q, Matthes E, Liao J, Radinovic S, Miyamoto C, Robert R, Hanrahan JW, Thomas DY.

Sci Rep. 2018 Jul 30;8(1):11404. doi: 10.1038/s41598-018-29276-y.


F508del-CFTR is not corrected by thymosin α1.

Matthes E, Hanrahan JW, Cantin AM.

Nat Med. 2018 Jul;24(7):890-891. doi: 10.1038/s41591-018-0079-6. No abstract available.


Most bicarbonate secretion by Calu-3 cells is mediated by CFTR and independent of pendrin.

Huang J, Kim D, Shan J, Abu-Arish A, Luo Y, Hanrahan JW.

Physiol Rep. 2018 Mar;6(5). doi: 10.14814/phy2.13641.


Velocity landscape correlation resolves multiple flowing protein populations from fluorescence image time series.

Pandžić E, Abu-Arish A, Whan RM, Hanrahan JW, Wiseman PW.

Methods. 2018 May 1;140-141:126-139. doi: 10.1016/j.ymeth.2018.02.011. Epub 2018 Feb 16.


Corrector combination therapies for F508del-CFTR.

Hanrahan JW, Matthes E, Carlile G, Thomas DY.

Curr Opin Pharmacol. 2017 Jun;34:105-111. doi: 10.1016/j.coph.2017.09.016. Epub 2017 Nov 5. Review.


Cigarette smoke activates CFTR through ROS-stimulated cAMP signaling in human bronchial epithelial cells.

Wong FH, AbuArish A, Matthes E, Turner MJ, Greene LE, Cloutier A, Robert R, Thomas DY, Cosa G, Cantin AM, Hanrahan JW.

Am J Physiol Cell Physiol. 2018 Jan 1;314(1):C118-C134. doi: 10.1152/ajpcell.00099.2017. Epub 2017 Oct 4.


Fenretinide differentially modulates the levels of long- and very long-chain ceramides by downregulating Cers5 enzyme: evidence from bench to bedside.

Garić D, De Sanctis JB, Wojewodka G, Houle D, Cupri S, Abu-Arish A, Hanrahan JW, Hajduch M, Matouk E, Radzioch D.

J Mol Med (Berl). 2017 Oct;95(10):1053-1064. doi: 10.1007/s00109-017-1564-y. Epub 2017 Jul 10.


Thymosin α1: a single drug with multiple targets in cystic fibrosis.

Cantin AM, Hanrahan JW.

Nat Med. 2017 May 5;23(5):536-538. doi: 10.1038/nm.4339. No abstract available.


Development of Automated Patch Clamp Technique to Investigate CFTR Chloride Channel Function.

Billet A, Froux L, Hanrahan JW, Becq F.

Front Pharmacol. 2017 Apr 7;8:195. doi: 10.3389/fphar.2017.00195. eCollection 2017.


PEGylated composite nanoparticles of PLGA and polyethylenimine for safe and efficient delivery of pDNA to lungs.

Kolte A, Patil S, Lesimple P, Hanrahan JW, Misra A.

Int J Pharm. 2017 May 30;524(1-2):382-396. doi: 10.1016/j.ijpharm.2017.03.094. Epub 2017 Apr 6.


β2-Adrenergic receptor agonists activate CFTR in intestinal organoids and subjects with cystic fibrosis.

Vijftigschild LA, Berkers G, Dekkers JF, Zomer-van Ommen DD, Matthes E, Kruisselbrink E, Vonk A, Hensen CE, Heida-Michel S, Geerdink M, Janssens HM, van de Graaf EA, Bronsveld I, de Winter-de Groot KM, Majoor CJ, Heijerman HG, de Jonge HR, Hanrahan JW, van der Ent CK, Beekman JM.

Eur Respir J. 2016 Sep;48(3):768-79. doi: 10.1183/13993003.01661-2015. Epub 2016 Jul 28.


Latonduine Analogs Restore F508del-Cystic Fibrosis Transmembrane Conductance Regulator Trafficking through the Modulation of Poly-ADP Ribose Polymerase 3 and Poly-ADP Ribose Polymerase 16 Activity.

Carlile GW, Robert R, Matthes E, Yang Q, Solari R, Hatley R, Edge CM, Hanrahan JW, Andersen R, Thomas DY, Birault V.

Mol Pharmacol. 2016 Aug;90(2):65-79. doi: 10.1124/mol.115.102418. Epub 2016 May 18.


Investigating CFTR and KCa3.1 Protein/Protein Interactions.

Klein H, Abu-Arish A, Trinh NT, Luo Y, Wiseman PW, Hanrahan JW, Brochiero E, Sauvé R.

PLoS One. 2016 Apr 19;11(4):e0153665. doi: 10.1371/journal.pone.0153665. eCollection 2016.


Potential sites of CFTR activation by tyrosine kinases.

Billet A, Jia Y, Jensen TJ, Hou YX, Chang XB, Riordan JR, Hanrahan JW.

Channels (Austin). 2016;10(3):247-51. doi: 10.1080/19336950.2015.1126010. Epub 2015 Dec 8.


The dual phosphodiesterase 3 and 4 inhibitor RPL554 stimulates CFTR and ciliary beating in primary cultures of bronchial epithelia.

Turner MJ, Matthes E, Billet A, Ferguson AJ, Thomas DY, Randell SH, Ostrowski LE, Abbott-Banner K, Hanrahan JW.

Am J Physiol Lung Cell Mol Physiol. 2016 Jan 1;310(1):L59-70. doi: 10.1152/ajplung.00324.2015. Epub 2015 Nov 6.


Low free drug concentration prevents inhibition of F508del CFTR functional expression by the potentiator VX-770 (ivacaftor).

Matthes E, Goepp J, Carlile GW, Luo Y, Dejgaard K, Billet A, Robert R, Thomas DY, Hanrahan JW.

Br J Pharmacol. 2016 Feb;173(3):459-70. doi: 10.1111/bph.13365. Epub 2016 Jan 13.


Cholesterol modulates CFTR confinement in the plasma membrane of primary epithelial cells.

Abu-Arish A, Pandzic E, Goepp J, Matthes E, Hanrahan JW, Wiseman PW.

Biophys J. 2015 Jul 7;109(1):85-94. doi: 10.1016/j.bpj.2015.04.042.


Regulation of the cystic fibrosis transmembrane conductance regulator anion channel by tyrosine phosphorylation.

Billet A, Jia Y, Jensen T, Riordan JR, Hanrahan JW.

FASEB J. 2015 Sep;29(9):3945-53. doi: 10.1096/fj.15-273151. Epub 2015 Jun 10.


Polymer assisted entrapment of netilmicin in PLGA nanoparticles for sustained antibacterial activity.

Kolate A, Kore G, Lesimple P, Baradia D, Patil S, Hanrahan JW, Misra A.

J Microencapsul. 2015;32(1):61-74. doi: 10.3109/02652048.2014.944951. Epub 2014 Sep 19.


Ibuprofen rescues mutant cystic fibrosis transmembrane conductance regulator trafficking.

Carlile GW, Robert R, Goepp J, Matthes E, Liao J, Kus B, Macknight SD, Rotin D, Hanrahan JW, Thomas DY.

J Cyst Fibros. 2015 Jan;14(1):16-25. doi: 10.1016/j.jcf.2014.06.001. Epub 2014 Jun 25.


The buffer capacity of airway epithelial secretions.

Kim D, Liao J, Hanrahan JW.

Front Physiol. 2014 Jun 3;5:188. doi: 10.3389/fphys.2014.00188. eCollection 2014.


The secret life of CFTR as a calcium-activated chloride channel.

Billet A, Hanrahan JW.

J Physiol. 2013 Nov 1;591(21):5273-8. doi: 10.1113/jphysiol.2013.261909. Epub 2013 Aug 19. Review.


Role of tyrosine phosphorylation in the muscarinic activation of the cystic fibrosis transmembrane conductance regulator (CFTR).

Billet A, Luo Y, Balghi H, Hanrahan JW.

J Biol Chem. 2013 Jul 26;288(30):21815-23. doi: 10.1074/jbc.M113.479360. Epub 2013 Jun 11.


Cystic fibrosis transmembrane conductance regulator is expressed in mucin granules from Calu-3 and primary human airway epithelial cells.

LeSimple P, Goepp J, Palmer ML, Fahrenkrug SC, O'Grady SM, Ferraro P, Robert R, Hanrahan JW.

Am J Respir Cell Mol Biol. 2013 Oct;49(4):511-6. doi: 10.1165/rcmb.2012-0419RC.


Novel pharmacological strategies to treat cystic fibrosis.

Hanrahan JW, Sampson HM, Thomas DY.

Trends Pharmacol Sci. 2013 Feb;34(2):119-25. doi: 10.1016/ Review.


Compounds that correct F508del-CFTR trafficking can also correct other protein trafficking diseases: an in vitro study using cell lines.

Sampson HM, Lam H, Chen PC, Zhang D, Mottillo C, Mirza M, Qasim K, Shrier A, Shyng SL, Hanrahan JW, Thomas DY.

Orphanet J Rare Dis. 2013 Jan 14;8:11. doi: 10.1186/1750-1172-8-11.


Correction of F508del-CFTR trafficking by the sponge alkaloid latonduine is modulated by interaction with PARP.

Carlile GW, Keyzers RA, Teske KA, Robert R, Williams DE, Linington RG, Gray CA, Centko RM, Yan L, Anjos SM, Sampson HM, Zhang D, Liao J, Hanrahan JW, Andersen RJ, Thomas DY.

Chem Biol. 2012 Oct 26;19(10):1288-99. doi: 10.1016/j.chembiol.2012.08.014.


Ouabain Mimics Low Temperature Rescue of F508del-CFTR in Cystic Fibrosis Epithelial Cells.

Zhang D, Ciciriello F, Anjos SM, Carissimo A, Liao J, Carlile GW, Balghi H, Robert R, Luini A, Hanrahan JW, Thomas DY.

Front Pharmacol. 2012 Oct 4;3:176. doi: 10.3389/fphar.2012.00176. eCollection 2012.


Decreasing Poly(ADP-Ribose) Polymerase Activity Restores ΔF508 CFTR Trafficking.

Anjos SM, Robert R, Waller D, Zhang DL, Balghi H, Sampson HM, Ciciriello F, Lesimple P, Carlile GW, Goepp J, Liao J, Ferraro P, Phillipe R, Dantzer F, Hanrahan JW, Thomas DY.

Front Pharmacol. 2012 Sep 12;3:165. doi: 10.3389/fphar.2012.00165. eCollection 2012.


Proinflammatory cytokine secretion is suppressed by TMEM16A or CFTR channel activity in human cystic fibrosis bronchial epithelia.

Veit G, Bossard F, Goepp J, Verkman AS, Galietta LJ, Hanrahan JW, Lukacs GL.

Mol Biol Cell. 2012 Nov;23(21):4188-202. doi: 10.1091/mbc.E12-06-0424. Epub 2012 Sep 12.


Basolateral chloride loading by the anion exchanger type 2: role in fluid secretion by the human airway epithelial cell line Calu-3.

Huang J, Shan J, Kim D, Liao J, Evagelidis A, Alper SL, Hanrahan JW.

J Physiol. 2012 Nov 1;590(21):5299-316. doi: 10.1113/jphysiol.2012.236919. Epub 2012 Jul 16.


Bicarbonate-dependent chloride transport drives fluid secretion by the human airway epithelial cell line Calu-3.

Shan J, Liao J, Huang J, Robert R, Palmer ML, Fahrenkrug SC, O'Grady SM, Hanrahan JW.

J Physiol. 2012 Nov 1;590(21):5273-97. doi: 10.1113/jphysiol.2012.236893. Epub 2012 Jul 9.


Physiology of epithelial chloride and fluid secretion.

Frizzell RA, Hanrahan JW.

Cold Spring Harb Perspect Med. 2012 Jun;2(6):a009563. doi: 10.1101/cshperspect.a009563.


Enhanced Ca2+ entry due to Orai1 plasma membrane insertion increases IL-8 secretion by cystic fibrosis airways.

Balghi H, Robert R, Rappaz B, Zhang X, Wohlhuter-Haddad A, Evagelidis A, Luo Y, Goepp J, Ferraro P, Roméo P, Trebak M, Wiseman PW, Thomas DY, Hanrahan JW.

FASEB J. 2011 Dec;25(12):4274-91. doi: 10.1096/fj.11-187682. Epub 2011 Aug 26.


Measurement of fluid secretion from intact airway submucosal glands.

Wine JJ, Joo NS, Choi JY, Cho HJ, Krouse ME, Wu JV, Khansaheb M, Irokawa T, Ianowski J, Hanrahan JW, Cuthbert AW, Tran KV.

Methods Mol Biol. 2011;742:93-112. doi: 10.1007/978-1-61779-120-8_6.


Identification of a NBD1-binding pharmacological chaperone that corrects the trafficking defect of F508del-CFTR.

Sampson HM, Robert R, Liao J, Matthes E, Carlile GW, Hanrahan JW, Thomas DY.

Chem Biol. 2011 Feb 25;18(2):231-42. doi: 10.1016/j.chembiol.2010.11.016.


Anion secretion by a model epithelium: more lessons from Calu-3.

Shan J, Huang J, Liao J, Robert R, Hanrahan JW.

Acta Physiol (Oxf). 2011 Jul;202(3):523-31. doi: 10.1111/j.1748-1716.2011.02253.x. Epub 2011 Mar 22. Review.


Correction of the Delta phe508 cystic fibrosis transmembrane conductance regulator trafficking defect by the bioavailable compound glafenine.

Robert R, Carlile GW, Liao J, Balghi H, Lesimple P, Liu N, Kus B, Rotin D, Wilke M, de Jonge HR, Scholte BJ, Thomas DY, Hanrahan JW.

Mol Pharmacol. 2010 Jun;77(6):922-30. doi: 10.1124/mol.109.062679. Epub 2010 Mar 3.


Cystic fibrosis transmembrane conductance regulator trafficking modulates the barrier function of airway epithelial cell monolayers.

LeSimple P, Liao J, Robert R, Gruenert DC, Hanrahan JW.

J Physiol. 2010 Apr 15;588(Pt 8):1195-209. doi: 10.1113/jphysiol.2009.182246. Epub 2010 Feb 15.


Lack of CFTR in skeletal muscle predisposes to muscle wasting and diaphragm muscle pump failure in cystic fibrosis mice.

Divangahi M, Balghi H, Danialou G, Comtois AS, Demoule A, Ernest S, Haston C, Robert R, Hanrahan JW, Radzioch D, Petrof BJ.

PLoS Genet. 2009 Jul;5(7):e1000586. doi: 10.1371/journal.pgen.1000586. Epub 2009 Jul 31.


Trafficking of immature DeltaF508-CFTR to the plasma membrane and its detection by biotinylation.

Luo Y, McDonald K, Hanrahan JW.

Biochem J. 2009 Apr 1;419(1):211-9, 2 p following 219. doi: 10.1042/BJ20081869.


PKC phosphorylation modulates PKA-dependent binding of the R domain to other domains of CFTR.

Seavilleklein G, Amer N, Evagelidis A, Chappe F, Irvine T, Hanrahan JW, Chappe V.

Am J Physiol Cell Physiol. 2008 Nov;295(5):C1366-75. doi: 10.1152/ajpcell.00034.2008. Epub 2008 Sep 17.


Role of the cystic fibrosis transmembrane conductance channel in human airway smooth muscle.

Michoud MC, Robert R, Hassan M, Moynihan B, Haston C, Govindaraju V, Ferraro P, Hanrahan JW, Martin JG.

Am J Respir Cell Mol Biol. 2009 Feb;40(2):217-22. doi: 10.1165/rcmb.2006-0444OC. Epub 2008 Aug 28.


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