Format
Sort by
Items per page

Send to

Choose Destination

Links from PubMed

Items: 1 to 20 of 120

1.

Functional stability of rescued delta F508 cystic fibrosis transmembrane conductance regulator in airway epithelial cells.

Jurkuvenaite A, Chen L, Bartoszewski R, Goldstein R, Bebok Z, Matalon S, Collawn JF.

Am J Respir Cell Mol Biol. 2010 Mar;42(3):363-72. doi: 10.1165/rcmb.2008-0434OC. Epub 2009 Jun 5.

2.

The short apical membrane half-life of rescued {Delta}F508-cystic fibrosis transmembrane conductance regulator (CFTR) results from accelerated endocytosis of {Delta}F508-CFTR in polarized human airway epithelial cells.

Swiatecka-Urban A, Brown A, Moreau-Marquis S, Renuka J, Coutermarsh B, Barnaby R, Karlson KH, Flotte TR, Fukuda M, Langford GM, Stanton BA.

J Biol Chem. 2005 Nov 4;280(44):36762-72. Epub 2005 Aug 30.

3.

Organic solutes rescue the functional defect in delta F508 cystic fibrosis transmembrane conductance regulator.

Zhang XM, Wang XT, Yue H, Leung SW, Thibodeau PH, Thomas PJ, Guggino SE.

J Biol Chem. 2003 Dec 19;278(51):51232-42. Epub 2003 Oct 7.

4.

Cystic fibrosis transmembrane conductance regulator trafficking modulates the barrier function of airway epithelial cell monolayers.

LeSimple P, Liao J, Robert R, Gruenert DC, Hanrahan JW.

J Physiol. 2010 Apr 15;588(Pt 8):1195-209. doi: 10.1113/jphysiol.2009.182246. Epub 2010 Feb 15.

5.

Nanomolar affinity small molecule correctors of defective Delta F508-CFTR chloride channel gating.

Yang H, Shelat AA, Guy RK, Gopinath VS, Ma T, Du K, Lukacs GL, Taddei A, Folli C, Pedemonte N, Galietta LJ, Verkman AS.

J Biol Chem. 2003 Sep 12;278(37):35079-85. Epub 2003 Jun 27.

6.

A delta F508 mutation in mouse cystic fibrosis transmembrane conductance regulator results in a temperature-sensitive processing defect in vivo.

French PJ, van Doorninck JH, Peters RH, Verbeek E, Ameen NA, Marino CR, de Jonge HR, Bijman J, Scholte BJ.

J Clin Invest. 1996 Sep 15;98(6):1304-12.

7.

A novel natural product compound enhances cAMP-regulated chloride conductance of cells expressing CFTR[delta]F508.

deCarvalho AC, Ndi CP, Tsopmo A, Tane P, Ayafor J, Connolly JD, Teem JL.

Mol Med. 2002 Feb;8(2):75-87.

8.

Transient receptor potential canonical channel 6 links Ca2+ mishandling to cystic fibrosis transmembrane conductance regulator channel dysfunction in cystic fibrosis.

Antigny F, Norez C, Dannhoffer L, Bertrand J, Raveau D, Corbi P, Jayle C, Becq F, Vandebrouck C.

Am J Respir Cell Mol Biol. 2011 Jan;44(1):83-90. doi: 10.1165/rcmb.2009-0347OC. Epub 2010 Mar 4.

PMID:
20203293
9.

Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature-sensitive.

Denning GM, Anderson MP, Amara JF, Marshall J, Smith AE, Welsh MJ.

Nature. 1992 Aug 27;358(6389):761-4.

PMID:
1380673
10.

Phenylglycine and sulfonamide correctors of defective delta F508 and G551D cystic fibrosis transmembrane conductance regulator chloride-channel gating.

Pedemonte N, Sonawane ND, Taddei A, Hu J, Zegarra-Moran O, Suen YF, Robins LI, Dicus CW, Willenbring D, Nantz MH, Kurth MJ, Galietta LJ, Verkman AS.

Mol Pharmacol. 2005 May;67(5):1797-807. Epub 2005 Feb 18.

11.

Chemical chaperones correct the mutant phenotype of the delta F508 cystic fibrosis transmembrane conductance regulator protein.

Brown CR, Hong-Brown LQ, Biwersi J, Verkman AS, Welch WJ.

Cell Stress Chaperones. 1996 Jun;1(2):117-25.

12.

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. doi: 10.1124/mol.115.099689. Epub 2015 Aug 5.

13.

Sequential quality-control checkpoints triage misfolded cystic fibrosis transmembrane conductance regulator.

Younger JM, Chen L, Ren HY, Rosser MF, Turnbull EL, Fan CY, Patterson C, Cyr DM.

Cell. 2006 Aug 11;126(3):571-82.

14.

Specific rescue of cystic fibrosis transmembrane conductance regulator processing mutants using pharmacological chaperones.

Wang Y, Bartlett MC, Loo TW, Clarke DM.

Mol Pharmacol. 2006 Jul;70(1):297-302. Epub 2006 Apr 19.

15.

∆F508 CFTR interactome remodelling promotes rescue of cystic fibrosis.

Pankow S, Bamberger C, Calzolari D, Martínez-Bartolomé S, Lavallée-Adam M, Balch WE, Yates JR 3rd.

Nature. 2015 Dec 24;528(7583):510-6. doi: 10.1038/nature15729. Epub 2015 Nov 30.

17.
18.
19.

The Delta F508 mutation shortens the biochemical half-life of plasma membrane CFTR in polarized epithelial cells.

Heda GD, Tanwani M, Marino CR.

Am J Physiol Cell Physiol. 2001 Jan;280(1):C166-74.

Supplemental Content

Support Center