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Results: 1 to 20 of 180

Related Citations for PubMed (Select 21303308)

1.

Cystic fibrosis transmembrane regulator potentiators as promising cystic fibrosis therapies.

Antoniu SA.

Expert Opin Investig Drugs. 2011 Mar;20(3):423-5. doi: 10.1517/13543784.2011.554823. Epub 2011 Feb 9.

PMID:
21303308
2.

Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation.

Accurso FJ, Rowe SM, Clancy JP, Boyle MP, Dunitz JM, Durie PR, Sagel SD, Hornick DB, Konstan MW, Donaldson SH, Moss RB, Pilewski JM, Rubenstein RC, Uluer AZ, Aitken ML, Freedman SD, Rose LM, Mayer-Hamblett N, Dong Q, Zha J, Stone AJ, Olson ER, Ordoñez CL, Campbell PW, Ashlock MA, Ramsey BW.

N Engl J Med. 2010 Nov 18;363(21):1991-2003. doi: 10.1056/NEJMoa0909825.

3.

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.

PMID:
22739718
4.

Ivacaftor treatment in patients with cystic fibrosis and the G551D-CFTR mutation.

Sermet-Gaudelus I.

Eur Respir Rev. 2013 Mar 1;22(127):66-71. doi: 10.1183/09059180.00008512. Review.

5.

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.

PMID:
23616952
6.

Optimizing nasal potential difference analysis for CFTR modulator development: assessment of ivacaftor in CF subjects with the G551D-CFTR mutation.

Rowe SM, Liu B, Hill A, Hathorne H, Cohen M, Beamer JR, Accurso FJ, Dong Q, Ordoñez CL, Stone AJ, Olson ER, Clancy JP; VX06-770-101 Study Group.

PLoS One. 2013 Jul 26;8(7):e66955. doi: 10.1371/journal.pone.0066955. Print 2013.

7.

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.

PMID:
24004658
8.

Ivacaftor in subjects with cystic fibrosis who are homozygous for the F508del-CFTR mutation.

Flume PA, Liou TG, Borowitz DS, Li H, Yen K, Ordoñez CL, Geller DE; VX 08-770-104 Study Group.

Chest. 2012 Sep;142(3):718-24.

9.

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.

10.

Efficacy and safety of ivacaftor in patients aged 6 to 11 years with cystic fibrosis with a G551D mutation.

Davies JC, Wainwright CE, Canny GJ, Chilvers MA, Howenstine MS, Munck A, Mainz JG, Rodriguez S, Li H, Yen K, Ordoñez CL, Ahrens R; VX08-770-103 (ENVISION) Study Group.

Am J Respir Crit Care Med. 2013 Jun 1;187(11):1219-25. doi: 10.1164/rccm.201301-0153OC.

11.

Managing the underlying cause of cystic fibrosis: a future role for potentiators and correctors.

Galietta LJ.

Paediatr Drugs. 2013 Oct;15(5):393-402. doi: 10.1007/s40272-013-0035-3. Review.

PMID:
23757197
12.

Targeting a genetic defect: cystic fibrosis transmembrane conductance regulator modulators in cystic fibrosis.

Derichs N.

Eur Respir Rev. 2013 Mar 1;22(127):58-65. doi: 10.1183/09059180.00008412. Review.

13.

Cystic fibrosis transmembrane conductance regulator dysfunction and its treatment.

Hull J.

J R Soc Med. 2012 Jun;105 Suppl 2:S2-8. doi: 10.1258/jrsm.2012.12s001. Review. No abstract available.

PMID:
22688363
14.

The impact of personalised therapies on respiratory medicine.

Elborn JS.

Eur Respir Rev. 2013 Mar 1;22(127):72-4. doi: 10.1183/09059180.00008212.

15.

Assessment of clinical response to ivacaftor with lung clearance index in cystic fibrosis patients with a G551D-CFTR mutation and preserved spirometry: a randomised controlled trial.

Davies J, Sheridan H, Bell N, Cunningham S, Davis SD, Elborn JS, Milla CE, Starner TD, Weiner DJ, Lee PS, Ratjen F.

Lancet Respir Med. 2013 Oct;1(8):630-8. doi: 10.1016/S2213-2600(13)70182-6. Epub 2013 Sep 10.

PMID:
24461666
16.

Regulatory domain phosphorylation to distinguish the mechanistic basis underlying acute CFTR modulators.

Pyle LC, Ehrhardt A, Mitchell LH, Fan L, Ren A, Naren AP, Li Y, Clancy JP, Bolger GB, Sorscher EJ, Rowe SM.

Am J Physiol Lung Cell Mol Physiol. 2011 Oct;301(4):L587-97. doi: 10.1152/ajplung.00465.2010. Epub 2011 Jul 1.

17.

Ivacaftor potentiation of multiple CFTR channels with gating mutations.

Yu H, Burton B, Huang CJ, Worley J, Cao D, Johnson JP Jr, Urrutia A, Joubran J, Seepersaud S, Sussky K, Hoffman BJ, Van Goor F.

J Cyst Fibros. 2012 May;11(3):237-45. doi: 10.1016/j.jcf.2011.12.005. Epub 2012 Jan 30.

18.

Cystic fibrosis transmembrane regulator correctors and potentiators.

Rowe SM, Verkman AS.

Cold Spring Harb Perspect Med. 2013 Jul 1;3(7). pii: a009761. doi: 10.1101/cshperspect.a009761. Review.

19.

A CFTR corrector (lumacaftor) and a CFTR potentiator (ivacaftor) for treatment of patients with cystic fibrosis who have a phe508del CFTR mutation: a phase 2 randomised controlled trial.

Boyle MP, Bell SC, Konstan MW, McColley SA, Rowe SM, Rietschel E, Huang X, Waltz D, Patel NR, Rodman D; VX09-809-102 study group.

Lancet Respir Med. 2014 Jul;2(7):527-38. doi: 10.1016/S2213-2600(14)70132-8. Epub 2014 Jun 24.

PMID:
24973281
20.

Results of a phase IIa study of VX-809, an investigational CFTR corrector compound, in subjects with cystic fibrosis homozygous for the F508del-CFTR mutation.

Clancy JP, Rowe SM, Accurso FJ, Aitken ML, Amin RS, Ashlock MA, Ballmann M, Boyle MP, Bronsveld I, Campbell PW, De Boeck K, Donaldson SH, Dorkin HL, Dunitz JM, Durie PR, Jain M, Leonard A, McCoy KS, Moss RB, Pilewski JM, Rosenbluth DB, Rubenstein RC, Schechter MS, Botfield M, Ordoñez CL, Spencer-Green GT, Vernillet L, Wisseh S, Yen K, Konstan MW.

Thorax. 2012 Jan;67(1):12-8. doi: 10.1136/thoraxjnl-2011-200393. Epub 2011 Aug 8.

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