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Results: 1 to 20 of 106

1.

Pharmacological rescue of the mutant cystic fibrosis transmembrane conductance regulator (CFTR) detected by use of a novel fluorescence platform.

Holleran JP, Glover ML, Peters KW, Bertrand CA, Watkins SC, Jarvik JW, Frizzell RA.

Mol Med. 2012 May 9;18:685-96. doi: 10.2119/molmed.2012.00001.

PMID:
22396015
[PubMed - indexed for MEDLINE]
Free PMC Article
2.

Probing conformational rescue induced by a chemical corrector of F508del-cystic fibrosis transmembrane conductance regulator (CFTR) mutant.

Yu W, Kim Chiaw P, Bear CE.

J Biol Chem. 2011 Jul 15;286(28):24714-25. doi: 10.1074/jbc.M111.239699. Epub 2011 May 21.

PMID:
21602569
[PubMed - indexed for MEDLINE]
Free PMC Article
3.

Regulated recycling of mutant CFTR is partially restored by pharmacological treatment.

Holleran JP, Zeng J, Frizzell RA, Watkins SC.

J Cell Sci. 2013 Jun 15;126(Pt 12):2692-703. doi: 10.1242/jcs.120196. Epub 2013 Apr 9.

PMID:
23572510
[PubMed - indexed for MEDLINE]
Free PMC Article
4.

Trimethylangelicin promotes the functional rescue of mutant F508del CFTR protein in cystic fibrosis airway cells.

Favia M, Mancini MT, Bezzerri V, Guerra L, Laselva O, Abbattiscianni AC, Debellis L, Reshkin SJ, Gambari R, Cabrini G, Casavola V.

Am J Physiol Lung Cell Mol Physiol. 2014 Jul 1;307(1):L48-61. doi: 10.1152/ajplung.00305.2013. Epub 2014 May 9.

PMID:
24816489
[PubMed - indexed for MEDLINE]
5.

Identification of synergistic combinations of F508del cystic fibrosis transmembrane conductance regulator (CFTR) modulators.

Lin S, Sui J, Cotard S, Fung B, Andersen J, Zhu P, El Messadi N, Lehar J, Lee M, Staunton J.

Assay Drug Dev Technol. 2010 Dec;8(6):669-84. doi: 10.1089/adt.2010.0313. Epub 2010 Nov 4.

PMID:
21050065
[PubMed - indexed for MEDLINE]
6.

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 15;108(46):18843-8. doi: 10.1073/pnas.1105787108. Epub 2011 Oct 5.

PMID:
21976485
[PubMed - indexed for MEDLINE]
Free PMC Article
7.

Correctors of the basic trafficking defect of the mutant F508del-CFTR that causes cystic fibrosis.

Birault V, Solari R, Hanrahan J, Thomas DY.

Curr Opin Chem Biol. 2013 Jun;17(3):353-60. doi: 10.1016/j.cbpa.2013.04.020. Epub 2013 May 24. Review.

PMID:
23711435
[PubMed - indexed for MEDLINE]
8.

Rescuing mutant CFTR: a multi-task approach to a better outcome in treating cystic fibrosis.

Amaral MD, Farinha CM.

Curr Pharm Des. 2013;19(19):3497-508. Review.

PMID:
23331027
[PubMed - indexed for MEDLINE]
9.

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.

PMID:
11139142
[PubMed - indexed for MEDLINE]
10.

A chemical corrector modifies the channel function of F508del-CFTR.

Kim Chiaw P, Wellhauser L, Huan LJ, Ramjeesingh M, Bear CE.

Mol Pharmacol. 2010 Sep;78(3):411-8. doi: 10.1124/mol.110.065862. Epub 2010 May 25.

PMID:
20501743
[PubMed - indexed for MEDLINE]
Free Article
11.

Control of cystic fibrosis transmembrane conductance regulator membrane trafficking: not just from the endoplasmic reticulum to the Golgi.

Farinha CM, Matos P, Amaral MD.

FEBS J. 2013 Sep;280(18):4396-406. doi: 10.1111/febs.12392. Epub 2013 Jul 5. Review.

PMID:
23773658
[PubMed - indexed for MEDLINE]
12.

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. Epub 2006 Jan 27.

PMID:
16443646
[PubMed - indexed for MEDLINE]
Free Article
13.

Targeting F508del-CFTR to develop rational new therapies for cystic fibrosis.

Cai ZW, Liu J, Li HY, Sheppard DN.

Acta Pharmacol Sin. 2011 Jun;32(6):693-701. doi: 10.1038/aps.2011.71. Review.

PMID:
21642944
[PubMed - indexed for MEDLINE]
Free PMC Article
14.

High-affinity activators of cystic fibrosis transmembrane conductance regulator (CFTR) chloride conductance identified by high-throughput screening.

Ma T, Vetrivel L, Yang H, Pedemonte N, Zegarra-Moran O, Galietta LJ, Verkman AS.

J Biol Chem. 2002 Oct 4;277(40):37235-41. Epub 2002 Aug 2.

PMID:
12161441
[PubMed - indexed for MEDLINE]
Free Article
15.

Revertant mutants G550E and 4RK rescue cystic fibrosis mutants in the first nucleotide-binding domain of CFTR by different mechanisms.

Roxo-Rosa M, Xu Z, Schmidt A, Neto M, Cai Z, Soares CM, Sheppard DN, Amaral MD.

Proc Natl Acad Sci U S A. 2006 Nov 21;103(47):17891-6. Epub 2006 Nov 10.

PMID:
17098864
[PubMed - indexed for MEDLINE]
Free PMC Article
16.

Rescue of the mutant CFTR chloride channel by pharmacological correctors and low temperature analyzed by gene expression profiling.

Sondo E, Tomati V, Caci E, Esposito AI, Pfeffer U, Pedemonte N, Galietta LJ.

Am J Physiol Cell Physiol. 2011 Oct;301(4):C872-85. doi: 10.1152/ajpcell.00507.2010. Epub 2011 Jul 13.

PMID:
21753184
[PubMed - indexed for MEDLINE]
Free PMC Article
17.

Resveratrol rescues cAMP-dependent anionic transport in the cystic fibrosis pancreatic cell line CFPAC1.

Hamdaoui N, Baudoin-Legros M, Kelly M, Aissat A, Moriceau S, Vieu DL, Colas J, Fritsch J, Edelman A, Planelles G.

Br J Pharmacol. 2011 Jun;163(4):876-86. doi: 10.1111/j.1476-5381.2011.01289.x.

PMID:
21366549
[PubMed - indexed for MEDLINE]
Free PMC Article
18.

Low temperature and chemical rescue affect molecular proximity of DeltaF508-cystic fibrosis transmembrane conductance regulator (CFTR) and epithelial sodium channel (ENaC).

Qadri YJ, Cormet-Boyaka E, Rooj AK, Lee W, Parpura V, Fuller CM, Berdiev BK.

J Biol Chem. 2012 May 11;287(20):16781-90. doi: 10.1074/jbc.M111.332031. Epub 2012 Mar 22.

PMID:
22442149
[PubMed - indexed for MEDLINE]
Free PMC Article
19.

Solubilizing mutations used to crystallize one CFTR domain attenuate the trafficking and channel defects caused by the major cystic fibrosis mutation.

Pissarra LS, Farinha CM, Xu Z, Schmidt A, Thibodeau PH, Cai Z, Thomas PJ, Sheppard DN, Amaral MD.

Chem Biol. 2008 Jan;15(1):62-9. doi: 10.1016/j.chembiol.2007.11.012.

PMID:
18215773
[PubMed - indexed for MEDLINE]
Free Article
20.

Proteasome-dependent pharmacological rescue of cystic fibrosis transmembrane conductance regulator revealed by mutation of glycine 622.

Norez C, Bilan F, Kitzis A, Mettey Y, Becq F.

J Pharmacol Exp Ther. 2008 Apr;325(1):89-99. doi: 10.1124/jpet.107.134502. Epub 2008 Jan 29.

PMID:
18230692
[PubMed - indexed for MEDLINE]
Free Article

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