333 related articles for article (PubMed ID: 26863533)
1. Potentiation of ΔF508- and G551D-CFTR-Mediated Cl- Current by Novel Hydroxypyrazolines.
Park J; Khloya P; Seo Y; Kumar S; Lee HK; Jeon DK; Jo S; Sharma PK; Namkung W
PLoS One; 2016; 11(2):e0149131. PubMed ID: 26863533
[TBL] [Abstract][Full Text] [Related]
2. 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]
3. Novel amino-carbonitrile-pyrazole identified in a small molecule screen activates wild-type and ΔF508 cystic fibrosis transmembrane conductance regulator in the absence of a cAMP agonist.
Namkung W; Park J; Seo Y; Verkman AS
Mol Pharmacol; 2013 Sep; 84(3):384-92. PubMed ID: 23788656
[TBL] [Abstract][Full Text] [Related]
4. Identification of natural coumarin compounds that rescue defective DeltaF508-CFTR chloride channel gating.
Xu LN; Na WL; Liu X; Hou SG; Lin S; Yang H; Ma TH
Clin Exp Pharmacol Physiol; 2008 Aug; 35(8):878-83. PubMed ID: 18430055
[TBL] [Abstract][Full Text] [Related]
5. Deletion of phenylalanine 508 causes attenuated phosphorylation-dependent activation of CFTR chloride channels.
Wang F; Zeltwanger S; Hu S; Hwang TC
J Physiol; 2000 May; 524 Pt 3(Pt 3):637-48. PubMed ID: 10790148
[TBL] [Abstract][Full Text] [Related]
6. Potentiators of Defective ΔF508-CFTR Gating that Do Not Interfere with Corrector Action.
Phuan PW; Veit G; Tan JA; Finkbeiner WE; Lukacs GL; Verkman AS
Mol Pharmacol; 2015 Oct; 88(4):791-9. PubMed ID: 26245207
[TBL] [Abstract][Full Text] [Related]
7. 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]
8. Cystic fibrosis transmembrane conductance regulator (CFTR) potentiators protect G551D but not ΔF508 CFTR from thermal instability.
Liu X; Dawson DC
Biochemistry; 2014 Sep; 53(35):5613-8. PubMed ID: 25148434
[TBL] [Abstract][Full Text] [Related]
9. Correction of the F508del-CFTR protein processing defect in vitro by the investigational drug VX-809.
Van Goor F; Hadida S; Grootenhuis PD; Burton B; Stack JH; Straley KS; Decker CJ; Miller M; McCartney J; Olson ER; Wine JJ; Frizzell RA; Ashlock M; Negulescu PA
Proc Natl Acad Sci U S A; 2011 Nov; 108(46):18843-8. PubMed ID: 21976485
[TBL] [Abstract][Full Text] [Related]
10. 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]
11. Rescue of DeltaF508-CFTR trafficking and gating in human cystic fibrosis airway primary cultures by small molecules.
Van Goor F; Straley KS; Cao D; González J; Hadida S; Hazlewood A; Joubran J; Knapp T; Makings LR; Miller M; Neuberger T; Olson E; Panchenko V; Rader J; Singh A; Stack JH; Tung R; Grootenhuis PD; Negulescu P
Am J Physiol Lung Cell Mol Physiol; 2006 Jun; 290(6):L1117-30. PubMed ID: 16443646
[TBL] [Abstract][Full Text] [Related]
12. Combination potentiator ('co-potentiator') therapy for CF caused by CFTR mutants, including N1303K, that are poorly responsive to single potentiators.
Phuan PW; Son JH; Tan JA; Li C; Musante I; Zlock L; Nielson DW; Finkbeiner WE; Kurth MJ; Galietta LJ; Haggie PM; Verkman AS
J Cyst Fibros; 2018 Sep; 17(5):595-606. PubMed ID: 29903467
[TBL] [Abstract][Full Text] [Related]
13. Synergy-based small-molecule screen using a human lung epithelial cell line yields ΔF508-CFTR correctors that augment VX-809 maximal efficacy.
Phuan PW; Veit G; Tan J; Roldan A; Finkbeiner WE; Lukacs GL; Verkman AS
Mol Pharmacol; 2014 Jul; 86(1):42-51. PubMed ID: 24737137
[TBL] [Abstract][Full Text] [Related]
14. 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]
15. Functional cystic fibrosis transmembrane conductance regulator tagged with an epitope of the vesicular stomatis virus glycoprotein can be addressed to the apical domain of polarized cells.
Costa de Beauregard MA; Edelman A; Chesnoy-Marchais D; Tondelier D; Lapillonne A; El Marjou F; Robine S; Louvard D
Eur J Cell Biol; 2000 Nov; 79(11):795-802. PubMed ID: 11139142
[TBL] [Abstract][Full Text] [Related]
16. 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]
17. NBD2 Is Required for the Rescue of Mutant F508del CFTR by a Thiazole-Based Molecule: A Class II Corrector for the Multi-Drug Therapy of Cystic Fibrosis.
Brandas C; Ludovico A; Parodi A; Moran O; Millo E; Cichero E; Baroni D
Biomolecules; 2021 Sep; 11(10):. PubMed ID: 34680050
[TBL] [Abstract][Full Text] [Related]
18. Potentiator synergy in rectal organoids carrying S1251N, G551D, or F508del CFTR mutations.
Dekkers JF; Van Mourik P; Vonk AM; Kruisselbrink E; Berkers G; de Winter-de Groot KM; Janssens HM; Bronsveld I; van der Ent CK; de Jonge HR; Beekman JM
J Cyst Fibros; 2016 Sep; 15(5):568-78. PubMed ID: 27160424
[TBL] [Abstract][Full Text] [Related]
19. 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]
20. Defective function of the cystic fibrosis-causing missense mutation G551D is recovered by genistein.
Illek B; Zhang L; Lewis NC; Moss RB; Dong JY; Fischer H
Am J Physiol; 1999 Oct; 277(4):C833-9. PubMed ID: 10516113
[TBL] [Abstract][Full Text] [Related]
[Next] [New Search]