These tools will no longer be maintained as of December 31, 2024. Archived website can be found here. PubMed4Hh GitHub repository can be found here. Contact NLM Customer Service if you have questions.


BIOMARKERS

Molecular Biopsy of Human Tumors

- a resource for Precision Medicine *

193 related articles for article (PubMed ID: 27707539)

  • 1. Therapeutic benefit observed with the CFTR potentiator, ivacaftor, in a CF patient homozygous for the W1282X CFTR nonsense mutation.
    Mutyam V; Libby EF; Peng N; Hadjiliadis D; Bonk M; Solomon GM; Rowe SM
    J Cyst Fibros; 2017 Jan; 16(1):24-29. PubMed ID: 27707539
    [TBL] [Abstract][Full Text] [Related]  

  • 2. Novel Correctors and Potentiators Enhance Translational Readthrough in CFTR Nonsense Mutations.
    Mutyam V; Sharma J; Li Y; Peng N; Chen J; Tang LP; Falk Libby E; Singh AK; Conrath K; Rowe SM
    Am J Respir Cell Mol Biol; 2021 May; 64(5):604-616. PubMed ID: 33616476
    [TBL] [Abstract][Full Text] [Related]  

  • 3. Synthetic aminoglycosides efficiently suppress cystic fibrosis transmembrane conductance regulator nonsense mutations and are enhanced by ivacaftor.
    Xue X; Mutyam V; Tang L; Biswas S; Du M; Jackson LA; Dai Y; Belakhov V; Shalev M; Chen F; Schacht J; J Bridges R; Baasov T; Hong J; Bedwell DM; Rowe SM
    Am J Respir Cell Mol Biol; 2014 Apr; 50(4):805-16. PubMed ID: 24251786
    [TBL] [Abstract][Full Text] [Related]  

  • 4. Ataluren/ivacaftor combination therapy: Two N-of-1 trials in cystic fibrosis patients with nonsense mutations.
    Peabody Lever JE; Mutyam V; Hathorne HY; Peng N; Sharma J; Edwards LJ; Rowe SM
    Pediatr Pulmonol; 2020 Jul; 55(7):1838-1842. PubMed ID: 32281737
    [TBL] [Abstract][Full Text] [Related]  

  • 5. Mutation-specific dual potentiators maximize rescue of CFTR gating mutants.
    Veit G; Da Fonte DF; Avramescu RG; Premchandar A; Bagdany M; Xu H; Bensinger D; Stubba D; Schmidt B; Matouk E; Lukacs GL
    J Cyst Fibros; 2020 Mar; 19(2):236-244. PubMed ID: 31678009
    [TBL] [Abstract][Full Text] [Related]  

  • 6. Resveratrol and ivacaftor are additive G551D CFTR-channel potentiators: therapeutic implications for cystic fibrosis sinus disease.
    Cho DY; Zhang S; Lazrak A; Grayson JW; Peña Garcia JA; Skinner DF; Lim DJ; Mackey C; Banks C; Matalon S; Woodworth BA
    Int Forum Allergy Rhinol; 2019 Jan; 9(1):100-105. PubMed ID: 30152192
    [TBL] [Abstract][Full Text] [Related]  

  • 7. Cystic fibrosis transmembrane conductance regulator-modifying medications: the future of cystic fibrosis treatment.
    Pettit RS
    Ann Pharmacother; 2012; 46(7-8):1065-75. PubMed ID: 22739718
    [TBL] [Abstract][Full Text] [Related]  

  • 8. In vivo and in vitro ivacaftor response in cystic fibrosis patients with residual CFTR function: N-of-1 studies.
    McGarry ME; Illek B; Ly NP; Zlock L; Olshansky S; Moreno C; Finkbeiner WE; Nielson DW
    Pediatr Pulmonol; 2017 Apr; 52(4):472-479. PubMed ID: 28068001
    [TBL] [Abstract][Full Text] [Related]  

  • 9. CFTR-dependent chloride efflux in cystic fibrosis mononuclear cells is increased by ivacaftor therapy.
    Guerra L; D'Oria S; Favia M; Castellani S; Santostasi T; Polizzi AM; Mariggiò MA; Gallo C; Casavola V; Montemurro P; Leonetti G; Manca A; Conese M
    Pediatr Pulmonol; 2017 Jul; 52(7):900-908. PubMed ID: 28445004
    [TBL] [Abstract][Full Text] [Related]  

  • 10. Nonsense-mediated RNA Decay Pathway Inhibition Restores Expression and Function of W1282X CFTR.
    Keenan MM; Huang L; Jordan NJ; Wong E; Cheng Y; Valley HC; Mahiou J; Liang F; Bihler H; Mense M; Guo S; Monia BP
    Am J Respir Cell Mol Biol; 2019 Sep; 61(3):290-300. PubMed ID: 30836009
    [TBL] [Abstract][Full Text] [Related]  

  • 11. Potentiators (specific therapies for class III and IV mutations) for cystic fibrosis.
    Skilton M; Krishan A; Patel S; Sinha IP; Southern KW
    Cochrane Database Syst Rev; 2019 Jan; 1(1):CD009841. PubMed ID: 30616300
    [TBL] [Abstract][Full Text] [Related]  

  • 12. 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;
    Chest; 2012 Sep; 142(3):718-724. PubMed ID: 22383668
    [TBL] [Abstract][Full Text] [Related]  

  • 13. Discovery of Clinically Approved Agents That Promote Suppression of Cystic Fibrosis Transmembrane Conductance Regulator Nonsense Mutations.
    Mutyam V; Du M; Xue X; Keeling KM; White EL; Bostwick JR; Rasmussen L; Liu B; Mazur M; Hong JS; Falk Libby E; Liang F; Shang H; Mense M; Suto MJ; Bedwell DM; Rowe SM
    Am J Respir Crit Care Med; 2016 Nov; 194(9):1092-1103. PubMed ID: 27104944
    [TBL] [Abstract][Full Text] [Related]  

  • 14. Effects of Lumacaftor-Ivacaftor Therapy on Cystic Fibrosis Transmembrane Conductance Regulator Function in Phe508del Homozygous Patients with Cystic Fibrosis.
    Graeber SY; Dopfer C; Naehrlich L; Gyulumyan L; Scheuermann H; Hirtz S; Wege S; Mairbäurl H; Dorda M; Hyde R; Bagheri-Hanson A; Rueckes-Nilges C; Fischer S; Mall MA; Tümmler B
    Am J Respir Crit Care Med; 2018 Jun; 197(11):1433-1442. PubMed ID: 29327948
    [TBL] [Abstract][Full Text] [Related]  

  • 15. Changes of CFTR functional measurements and clinical improvements in cystic fibrosis patients with non p.Gly551Asp gating mutations treated with ivacaftor.
    Mesbahi M; Shteinberg M; Wilschanski M; Hatton A; Nguyen-Khoa T; Friedman H; Cohen M; Escabasse V; Le Bourgeois M; Lucidi V; Sermet-Gaudelus I; Bassinet L; Livnat G
    J Cyst Fibros; 2017 Jan; 16(1):45-48. PubMed ID: 27659740
    [TBL] [Abstract][Full Text] [Related]  

  • 16. Correctors and Potentiators Rescue Function of the Truncated W1282X-Cystic Fibrosis Transmembrane Regulator (CFTR) Translation Product.
    Haggie PM; Phuan PW; Tan JA; Xu H; Avramescu RG; Perdomo D; Zlock L; Nielson DW; Finkbeiner WE; Lukacs GL; Verkman AS
    J Biol Chem; 2017 Jan; 292(3):771-785. PubMed ID: 27895116
    [TBL] [Abstract][Full Text] [Related]  

  • 17. VX-445-Tezacaftor-Ivacaftor in Patients with Cystic Fibrosis and One or Two Phe508del Alleles.
    Keating D; Marigowda G; Burr L; Daines C; Mall MA; McKone EF; Ramsey BW; Rowe SM; Sass LA; Tullis E; McKee CM; Moskowitz SM; Robertson S; Savage J; Simard C; Van Goor F; Waltz D; Xuan F; Young T; Taylor-Cousar JL;
    N Engl J Med; 2018 Oct; 379(17):1612-1620. PubMed ID: 30334692
    [TBL] [Abstract][Full Text] [Related]  

  • 18. 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;
    Am J Respir Crit Care Med; 2014 Jul; 190(2):175-84. PubMed ID: 24927234
    [TBL] [Abstract][Full Text] [Related]  

  • 19. Ivacaftor potentiation of multiple CFTR channels with gating mutations.
    Yu H; Burton B; Huang CJ; Worley J; Cao D; Johnson JP; Urrutia A; Joubran J; Seepersaud S; Sussky K; Hoffman BJ; Van Goor F
    J Cyst Fibros; 2012 May; 11(3):237-45. PubMed ID: 22293084
    [TBL] [Abstract][Full Text] [Related]  

  • 20. Suppression of CFTR premature termination codons and rescue of CFTR protein and function by the synthetic aminoglycoside NB54.
    Rowe SM; Sloane P; Tang LP; Backer K; Mazur M; Buckley-Lanier J; Nudelman I; Belakhov V; Bebok Z; Schwiebert E; Baasov T; Bedwell DM
    J Mol Med (Berl); 2011 Nov; 89(11):1149-61. PubMed ID: 21779978
    [TBL] [Abstract][Full Text] [Related]  

    [Next]    [New Search]
    of 10.