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787 related items for PubMed ID: 25763566
1. Impact of the F508del mutation on ovine CFTR, a Cl- channel with enhanced conductance and ATP-dependent gating. Cai Z, Palmai-Pallag T, Khuituan P, Mutolo MJ, Boinot C, Liu B, Scott-Ward TS, Callebaut I, Harris A, Sheppard DN. J Physiol; 2015 Jun 01; 593(11):2427-46. PubMed ID: 25763566 [Abstract] [Full Text] [Related]
2. 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 01; 317(1):L71-L86. PubMed ID: 30969810 [Abstract] [Full Text] [Related]
3. 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 15; 594(12):3227-44. PubMed ID: 26846474 [Abstract] [Full Text] [Related]
4. Altering intracellular pH reveals the kinetic basis of intraburst gating in the CFTR Cl- channel. Chen JH, Xu W, Sheppard DN. J Physiol; 2017 Feb 15; 595(4):1059-1076. PubMed ID: 27779763 [Abstract] [Full Text] [Related]
5. 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 03; 292(9):3706-3719. PubMed ID: 28087700 [Abstract] [Full Text] [Related]
6. Potentiation of cystic fibrosis transmembrane conductance regulator (CFTR) Cl- currents by the chemical solvent tetrahydrofuran. Hughes LK, Ju M, Sheppard DN. Mol Membr Biol; 2008 Sep 03; 25(6-7):528-38. PubMed ID: 18989824 [Abstract] [Full Text] [Related]
7. 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 03; 171(19):4490-503. PubMed ID: 24902474 [Abstract] [Full Text] [Related]
8. Exploiting species differences to understand the CFTR Cl- channel. Bose SJ, Scott-Ward TS, Cai Z, Sheppard DN. Biochem Soc Trans; 2015 Oct 03; 43(5):975-82. PubMed ID: 26517912 [Abstract] [Full Text] [Related]
9. 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 03; 597(2):543-560. PubMed ID: 30408177 [Abstract] [Full Text] [Related]
10. 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 03; 179(7):1319-1337. PubMed ID: 34644413 [Abstract] [Full Text] [Related]
12. Differential sensitivity of the cystic fibrosis (CF)-associated mutants G551D and G1349D to potentiators of the cystic fibrosis transmembrane conductance regulator (CFTR) Cl- channel. Cai Z, Taddei A, Sheppard DN. J Biol Chem; 2006 Jan 27; 281(4):1970-7. PubMed ID: 16311240 [Abstract] [Full Text] [Related]
13. Potentiation of the cystic fibrosis transmembrane conductance regulator Cl- channel by ivacaftor is temperature independent. Wang Y, Cai Z, Gosling M, Sheppard DN. Am J Physiol Lung Cell Mol Physiol; 2018 Nov 01; 315(5):L846-L857. PubMed ID: 30136610 [Abstract] [Full Text] [Related]
14. Partial rescue of F508del-cystic fibrosis transmembrane conductance regulator channel gating with modest improvement of protein processing, but not stability, by a dual-acting small molecule. Liu J, Bihler H, Farinha CM, Awatade NT, Romão AM, Mercadante D, Cheng Y, Musisi I, Jantarajit W, Wang Y, Cai Z, Amaral MD, Mense M, Sheppard DN. Br J Pharmacol; 2018 Apr 01; 175(7):1017-1038. PubMed ID: 29318594 [Abstract] [Full Text] [Related]
18. CFTR gating I: Characterization of the ATP-dependent gating of a phosphorylation-independent CFTR channel (DeltaR-CFTR). Bompadre SG, Ai T, Cho JH, Wang X, Sohma Y, Li M, Hwang TC. J Gen Physiol; 2005 Apr 01; 125(4):361-75. PubMed ID: 15767295 [Abstract] [Full Text] [Related]