1027 related articles for article (PubMed ID: 30408177)
1. 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]
2. 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]
3. Physiological and pharmacological characterization of the N1303K mutant CFTR.
DeStefano S; Gees M; Hwang TC
J Cyst Fibros; 2018 Sep; 17(5):573-581. PubMed ID: 29887518
[TBL] [Abstract][Full Text] [Related]
4. 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]
5. 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]
6. 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]
7. 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]
8. 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]
9. G551D and G1349D, two CF-associated mutations in the signature sequences of CFTR, exhibit distinct gating defects.
Bompadre SG; Sohma Y; Li M; Hwang TC
J Gen Physiol; 2007 Apr; 129(4):285-98. PubMed ID: 17353351
[TBL] [Abstract][Full Text] [Related]
10. A common mechanism for CFTR potentiators.
Yeh HI; Sohma Y; Conrath K; Hwang TC
J Gen Physiol; 2017 Dec; 149(12):1105-1118. PubMed ID: 29079713
[TBL] [Abstract][Full Text] [Related]
11. 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. PubMed ID: 26416827
[TBL] [Abstract][Full Text] [Related]
12. 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]
13. 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]
14. 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]
15. Vx-770 potentiates CFTR function by promoting decoupling between the gating cycle and ATP hydrolysis cycle.
Jih KY; Hwang TC
Proc Natl Acad Sci U S A; 2013 Mar; 110(11):4404-9. PubMed ID: 23440202
[TBL] [Abstract][Full Text] [Related]
16. Potentiation of the cystic fibrosis transmembrane conductance regulator by VX-770 involves stabilization of the pre-hydrolytic, O
Langron E; Prins S; Vergani P
Br J Pharmacol; 2018 Oct; 175(20):3990-4002. PubMed ID: 30107029
[TBL] [Abstract][Full Text] [Related]
17. The most common cystic fibrosis-associated mutation destabilizes the dimeric state of the nucleotide-binding domains of CFTR.
Jih KY; Li M; Hwang TC; Bompadre SG
J Physiol; 2011 Jun; 589(Pt 11):2719-31. PubMed ID: 21486785
[TBL] [Abstract][Full Text] [Related]
18. 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; 6(246):246ra97. PubMed ID: 25101887
[TBL] [Abstract][Full Text] [Related]
19. Mechanistic Approaches to Improve Correction of the Most Common Disease-Causing Mutation in Cystic Fibrosis.
Bali V; Lazrak A; Guroji P; Matalon S; Bebok Z
PLoS One; 2016; 11(5):e0155882. PubMed ID: 27214033
[TBL] [Abstract][Full Text] [Related]
20. 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]
[Next] [New Search]
