BIOMARKERS

Molecular Biopsy of Human Tumors

- a resource for Precision Medicine *

415 related articles for article (PubMed ID: 30136610)

  • 1. Potentiation of the cystic fibrosis transmembrane conductance regulator Cl
    Wang Y; Cai Z; Gosling M; Sheppard DN
    Am J Physiol Lung Cell Mol Physiol; 2018 Nov; 315(5):L846-L857. PubMed ID: 30136610
    [TBL] [Abstract][Full Text] [Related]  

  • 2. Two Small Molecules Restore Stability to a Subpopulation of the Cystic Fibrosis Transmembrane Conductance Regulator with the Predominant Disease-causing Mutation.
    Meng X; Wang Y; Wang X; Wrennall JA; Rimington TL; Li H; Cai Z; Ford RC; Sheppard DN
    J Biol Chem; 2017 Mar; 292(9):3706-3719. PubMed ID: 28087700
    [TBL] [Abstract][Full Text] [Related]  

  • 3. Differential thermostability and response to cystic fibrosis transmembrane conductance regulator potentiators of human and mouse F508del-CFTR.
    Bose SJ; Bijvelds MJC; Wang Y; Liu J; Cai Z; Bot AGM; de Jonge HR; Sheppard DN
    Am J Physiol Lung Cell Mol Physiol; 2019 Jul; 317(1):L71-L86. PubMed ID: 30969810
    [TBL] [Abstract][Full Text] [Related]  

  • 4. Two rare variants that affect the same amino acid in CFTR have distinct responses to ivacaftor.
    Li H; Rodrat M; Al-Salmani MK; Veselu DF; Han ST; Raraigh KS; Cutting GR; Sheppard DN
    J Physiol; 2024 Jan; 602(2):333-354. PubMed ID: 38186087
    [TBL] [Abstract][Full Text] [Related]  

  • 5. A small molecule CFTR potentiator restores ATP-dependent channel gating to the cystic fibrosis mutant G551D-CFTR.
    Liu J; Berg AP; Wang Y; Jantarajit W; Sutcliffe KJ; Stevens EB; Cao L; Pregel MJ; Sheppard DN
    Br J Pharmacol; 2022 Apr; 179(7):1319-1337. PubMed ID: 34644413
    [TBL] [Abstract][Full Text] [Related]  

  • 6. CFTR potentiators partially restore channel function to A561E-CFTR, a cystic fibrosis mutant with a similar mechanism of dysfunction as F508del-CFTR.
    Wang Y; Liu J; Loizidou A; Bugeja LA; Warner R; Hawley BR; Cai Z; Toye AM; Sheppard DN; Li H
    Br J Pharmacol; 2014 Oct; 171(19):4490-503. PubMed ID: 24902474
    [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. On the mechanism of gating defects caused by the R117H mutation in cystic fibrosis transmembrane conductance regulator.
    Yu YC; Sohma Y; Hwang TC
    J Physiol; 2016 Jun; 594(12):3227-44. PubMed ID: 26846474
    [TBL] [Abstract][Full Text] [Related]  

  • 9. Synergistic Potentiation of Cystic Fibrosis Transmembrane Conductance Regulator Gating by Two Chemically Distinct Potentiators, Ivacaftor (VX-770) and 5-Nitro-2-(3-Phenylpropylamino) Benzoate.
    Lin WY; Sohma Y; Hwang TC
    Mol Pharmacol; 2016 Sep; 90(3):275-85. PubMed ID: 27413118
    [TBL] [Abstract][Full Text] [Related]  

  • 10. Structural mechanisms for defective CFTR gating caused by the Q1412X mutation, a severe Class VI pathogenic mutation in cystic fibrosis.
    Yeh JT; Yu YC; Hwang TC
    J Physiol; 2019 Jan; 597(2):543-560. PubMed ID: 30408177
    [TBL] [Abstract][Full Text] [Related]  

  • 11. ORKAMBI-Mediated Rescue of Mucociliary Clearance in Cystic Fibrosis Primary Respiratory Cultures Is Enhanced by Arginine Uptake, Arginase Inhibition, and Promotion of Nitric Oxide Signaling to the Cystic Fibrosis Transmembrane Conductance Regulator Channel.
    Wu YS; Jiang J; Ahmadi S; Lew A; Laselva O; Xia S; Bartlett C; Ip W; Wellhauser L; Ouyang H; Gonska T; Moraes TJ; Bear CE
    Mol Pharmacol; 2019 Oct; 96(4):515-525. PubMed ID: 31427400
    [TBL] [Abstract][Full Text] [Related]  

  • 12. Extracellular phosphate enhances the function of F508del-CFTR rescued by CFTR correctors.
    Saint-Criq V; Wang Y; Delpiano L; Lin J; Sheppard DN; Gray MA
    J Cyst Fibros; 2021 Sep; 20(5):843-850. PubMed ID: 34020896
    [TBL] [Abstract][Full Text] [Related]  

  • 13. 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. PubMed ID: 26492939
    [TBL] [Abstract][Full Text] [Related]  

  • 14. 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]  

  • 15. 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. PubMed ID: 29903751
    [TBL] [Abstract][Full Text] [Related]  

  • 16. Restoration of R117H CFTR folding and function in human airway cells through combination treatment with VX-809 and VX-770.
    Gentzsch M; Ren HY; Houck SA; Quinney NL; Cholon DM; Sopha P; Chaudhry IG; Das J; Dokholyan NV; Randell SH; Cyr DM
    Am J Physiol Lung Cell Mol Physiol; 2016 Sep; 311(3):L550-9. PubMed ID: 27402691
    [TBL] [Abstract][Full Text] [Related]  

  • 17. Cystic fibrosis drug ivacaftor stimulates CFTR channels at picomolar concentrations.
    Csanády L; Töröcsik B
    Elife; 2019 Jun; 8():. PubMed ID: 31205003
    [TBL] [Abstract][Full Text] [Related]  

  • 18. Lumacaftor alone and combined with ivacaftor: preclinical and clinical trial experience of F508del CFTR correction.
    Brewington JJ; McPhail GL; Clancy JP
    Expert Rev Respir Med; 2016; 10(1):5-17. PubMed ID: 26581802
    [TBL] [Abstract][Full Text] [Related]  

  • 19. 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. PubMed ID: 27585394
    [TBL] [Abstract][Full Text] [Related]  

  • 20. Effect of ivacaftor on CFTR forms with missense mutations associated with defects in protein processing or function.
    Van Goor F; Yu H; Burton B; Hoffman BJ
    J Cyst Fibros; 2014 Jan; 13(1):29-36. PubMed ID: 23891399
    [TBL] [Abstract][Full Text] [Related]  

    [Next]    [New Search]
    of 21.