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A pharmacologic approach to acquired cystic fibrosis transmembrane conductance regulator dysfunction in smoking related lung disease.

Sloane PA, Shastry S, Wilhelm A, Courville C, Tang LP, Backer K, Levin E, Raju SV, Li Y, Mazur M, Byan-Parker S, Grizzle W, Sorscher EJ, Dransfield MT, Rowe SM.

PLoS One. 2012;7(6):e39809. doi: 10.1371/journal.pone.0039809. Epub 2012 Jun 29.


Cystic fibrosis transmembrane conductance regulator activation by roflumilast contributes to therapeutic benefit in chronic bronchitis.

Lambert JA, Raju SV, Tang LP, McNicholas CM, Li Y, Courville CA, Farris RF, Coricor GE, Smoot LH, Mazur MM, Dransfield MT, Bolger GB, Rowe SM.

Am J Respir Cell Mol Biol. 2014 Mar;50(3):549-58. doi: 10.1165/rcmb.2013-0228OC.


Resveratrol ameliorates abnormalities of fluid and electrolyte secretion in a hypoxia-Induced model of acquired CFTR deficiency.

Woodworth BA.

Laryngoscope. 2015 Oct;125 Suppl 7:S1-S13. doi: 10.1002/lary.25335. Epub 2015 May 6.


The Cystic Fibrosis Transmembrane Conductance Regulator Potentiator Ivacaftor Augments Mucociliary Clearance Abrogating Cystic Fibrosis Transmembrane Conductance Regulator Inhibition by Cigarette Smoke.

Raju SV, Lin VY, Liu L, McNicholas CM, Karki S, Sloane PA, Tang L, Jackson PL, Wang W, Wilson L, Macon KJ, Mazur M, Kappes JC, DeLucas LJ, Barnes S, Kirk K, Tearney GJ, Rowe SM.

Am J Respir Cell Mol Biol. 2017 Jan;56(1):99-108. doi: 10.1165/rcmb.2016-0226OC.


Potentiators (specific therapies for class III and IV mutations) for cystic fibrosis.

Patel S, Sinha IP, Dwan K, Echevarria C, Schechter M, Southern KW.

Cochrane Database Syst Rev. 2015 Mar 26;(3):CD009841. doi: 10.1002/14651858.CD009841.pub2. Review.


Acquired cystic fibrosis transmembrane conductance regulator dysfunction in the lower airways in COPD.

Dransfield MT, Wilhelm AM, Flanagan B, Courville C, Tidwell SL, Raju SV, Gaggar A, Steele C, Tang LP, Liu B, Rowe SM.

Chest. 2013 Aug;144(2):498-506. doi: 10.1378/chest.13-0274.


Some gating potentiators, including VX-770, diminish ΔF508-CFTR functional expression.

Veit G, Avramescu RG, Perdomo D, Phuan PW, Bagdany M, Apaja PM, Borot F, Szollosi D, Wu YS, Finkbeiner WE, Hegedus T, Verkman AS, Lukacs GL.

Sci Transl Med. 2014 Jul 23;6(246):246ra97. doi: 10.1126/scitranslmed.3008889.


Clinical mechanism of the cystic fibrosis transmembrane conductance regulator potentiator ivacaftor in G551D-mediated cystic fibrosis.

Rowe SM, Heltshe SL, Gonska T, Donaldson SH, Borowitz D, Gelfond D, Sagel SD, Khan U, Mayer-Hamblett N, Van Dalfsen JM, Joseloff E, Ramsey BW; GOAL Investigators of the Cystic Fibrosis Foundation Therapeutics Development Network..

Am J Respir Crit Care Med. 2014 Jul 15;190(2):175-84. doi: 10.1164/rccm.201404-0703OC.


Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770.

Van Goor F, Hadida S, Grootenhuis PD, Burton B, Cao D, Neuberger T, Turnbull A, Singh A, Joubran J, Hazlewood A, Zhou J, McCartney J, Arumugam V, Decker C, Yang J, Young C, Olson ER, Wine JJ, Frizzell RA, Ashlock M, Negulescu P.

Proc Natl Acad Sci U S A. 2009 Nov 3;106(44):18825-30. doi: 10.1073/pnas.0904709106. Epub 2009 Oct 21.


Resveratrol enhances airway surface liquid depth in sinonasal epithelium by increasing cystic fibrosis transmembrane conductance regulator open probability.

Zhang S, Blount AC, McNicholas CM, Skinner DF, Chestnut M, Kappes JC, Sorscher EJ, Woodworth BA.

PLoS One. 2013 Nov 25;8(11):e81589. doi: 10.1371/journal.pone.0081589. eCollection 2013. Erratum in: PLoS One. 2014;9(1). doi:10.1371/annotation/d852ff1d-2824-4f4d-80f2-9be7a42a5f25.


Potentiator ivacaftor abrogates pharmacological correction of ΔF508 CFTR in cystic fibrosis.

Cholon DM, Quinney NL, Fulcher ML, Esther CR Jr, Das J, Dokholyan NV, Randell SH, Boucher RC, Gentzsch M.

Sci Transl Med. 2014 Jul 23;6(246):246ra96. doi: 10.1126/scitranslmed.3008680.


Cigarette smoke induces systemic defects in cystic fibrosis transmembrane conductance regulator function.

Raju SV, Jackson PL, Courville CA, McNicholas CM, Sloane PA, Sabbatini G, Tidwell S, Tang LP, Liu B, Fortenberry JA, Jones CW, Boydston JA, Clancy JP, Bowen LE, Accurso FJ, Blalock JE, Dransfield MT, Rowe SM.

Am J Respir Crit Care Med. 2013 Dec 1;188(11):1321-30. doi: 10.1164/rccm.201304-0733OC.


Transforming growth factor-β1 and cigarette smoke inhibit the ability of β2-agonists to enhance epithelial permeability.

Unwalla HJ, Ivonnet P, Dennis JS, Conner GE, Salathe M.

Am J Respir Cell Mol Biol. 2015 Jan;52(1):65-74. doi: 10.1165/rcmb.2013-0538OC.


Cystic fibrosis transmembrane conductance regulator activation by the solvent ethanol: implications for topical drug delivery.

Cho DY, Skinner D, Zhang S, Fortenberry J, Sorscher EJ, Dean NR, Woodworth BA.

Int Forum Allergy Rhinol. 2016 Feb;6(2):178-84. doi: 10.1002/alr.21638. Epub 2015 Dec 3.


Roflumilast partially reverses smoke-induced mucociliary dysfunction.

Schmid A, Baumlin N, Ivonnet P, Dennis JS, Campos M, Krick S, Salathe M.

Respir Res. 2015 Oct 31;16:135. doi: 10.1186/s12931-015-0294-3.


A CFTR potentiator in patients with cystic fibrosis and the G551D mutation.

Ramsey BW, Davies J, McElvaney NG, Tullis E, Bell SC, Dřevínek P, Griese M, McKone EF, Wainwright CE, Konstan MW, Moss R, Ratjen F, Sermet-Gaudelus I, Rowe SM, Dong Q, Rodriguez S, Yen K, Ordoñez C, Elborn JS; VX08-770-102 Study Group..

N Engl J Med. 2011 Nov 3;365(18):1663-72. doi: 10.1056/NEJMoa1105185.


Cystic fibrosis transmembrane conductance regulator-modifying medications: the future of cystic fibrosis treatment.

Pettit RS.

Ann Pharmacother. 2012 Jul-Aug;46(7-8):1065-75. doi: 10.1345/aph.1R076. Epub 2012 Jun 26. Review.


Lumacaftor and ivacaftor in the management of patients with cystic fibrosis: current evidence and future prospects.

Kuk K, Taylor-Cousar JL.

Ther Adv Respir Dis. 2015 Dec;9(6):313-26. doi: 10.1177/1753465815601934. Epub 2015 Sep 28. Review.


Ivacaftor: the first therapy acting on the primary cause of cystic fibrosis.

McPhail GL, Clancy JP.

Drugs Today (Barc). 2013 Apr;49(4):253-60. doi: 10.1358/dot.2013.49.4.1940984.


Ivacaftor treatment of cystic fibrosis patients with the G551D mutation: a review of the evidence.

Kotha K, Clancy JP.

Ther Adv Respir Dis. 2013 Oct;7(5):288-96. doi: 10.1177/1753465813502115. Epub 2013 Sep 3. Review.


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