826 related articles for article (PubMed ID: 27585394)
21. 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]
22. 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]
23. Cigarette Smoke-Induced Acquired Dysfunction of Cystic Fibrosis Transmembrane Conductance Regulator in the Pathogenesis of Chronic Obstructive Pulmonary Disease.
Shi J; Li H; Yuan C; Luo M; Wei J; Liu X
Oxid Med Cell Longev; 2018; 2018():6567578. PubMed ID: 29849907
[TBL] [Abstract][Full Text] [Related]
24. 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; 314(1):C118-C134. PubMed ID: 28978522
[TBL] [Abstract][Full Text] [Related]
25. 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]
26. 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. PubMed ID: 24978189
[TBL] [Abstract][Full Text] [Related]
27. Potentiator ivacaftor abrogates pharmacological correction of ΔF508 CFTR in cystic fibrosis.
Cholon DM; Quinney NL; Fulcher ML; Esther CR; Das J; Dokholyan NV; Randell SH; Boucher RC; Gentzsch M
Sci Transl Med; 2014 Jul; 6(246):246ra96. PubMed ID: 25101886
[TBL] [Abstract][Full Text] [Related]
28. 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]
29. 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]
30. 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]
31. Mutation-specific downregulation of CFTR2 variants by gating potentiators.
Avramescu RG; Kai Y; Xu H; Bidaud-Meynard A; Schnúr A; Frenkiel S; Matouk E; Veit G; Lukacs GL
Hum Mol Genet; 2017 Dec; 26(24):4873-4885. PubMed ID: 29040544
[TBL] [Abstract][Full Text] [Related]
32. 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]
33. 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]
34. Cystic Fibrosis Transmembrane Conductance Regulator Potentiation as a Therapeutic Strategy for Pulmonary Edema: A Proof-of-Concept Study in Pigs.
Li X; Vargas Buonfiglio LG; Adam RJ; Stoltz DA; Zabner J; Comellas AP
Crit Care Med; 2017 Dec; 45(12):e1240-e1246. PubMed ID: 28953499
[TBL] [Abstract][Full Text] [Related]
35. Cystic fibrosis transmembrane conductance regulator (CFTR) potentiator VX-770 (ivacaftor) opens the defective channel gate of mutant CFTR in a phosphorylation-dependent but ATP-independent manner.
Eckford PD; Li C; Ramjeesingh M; Bear CE
J Biol Chem; 2012 Oct; 287(44):36639-49. PubMed ID: 22942289
[TBL] [Abstract][Full Text] [Related]
36. 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; 188(11):1321-30. PubMed ID: 24040746
[TBL] [Abstract][Full Text] [Related]
37. Antisense oligonucleotide-mediated correction of CFTR splicing improves chloride secretion in cystic fibrosis patient-derived bronchial epithelial cells.
Michaels WE; Bridges RJ; Hastings ML
Nucleic Acids Res; 2020 Jul; 48(13):7454-7467. PubMed ID: 32520327
[TBL] [Abstract][Full Text] [Related]
38. TMEM16A Potentiation: A Novel Therapeutic Approach for the Treatment of Cystic Fibrosis.
Danahay HL; Lilley S; Fox R; Charlton H; Sabater J; Button B; McCarthy C; Collingwood SP; Gosling M
Am J Respir Crit Care Med; 2020 Apr; 201(8):946-954. PubMed ID: 31898911
[No Abstract] [Full Text] [Related]
39. Cystic fibrosis transmembrane conductance regulator (CFTR) modulators have differential effects on cystic fibrosis macrophage function.
Zhang S; Shrestha CL; Kopp BT
Sci Rep; 2018 Nov; 8(1):17066. PubMed ID: 30459435
[TBL] [Abstract][Full Text] [Related]
40. 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]
[Previous] [Next] [New Search]
