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Items: 1 to 20 of 384

1.

The ΔF508-CFTR mutation inhibits wild-type CFTR processing and function when co-expressed in human airway epithelia and in mouse nasal mucosa.

Tucker TA, Fortenberry JA, Zsembery A, Schwiebert LM, Schwiebert EM.

BMC Physiol. 2012 Sep 24;12:12. doi: 10.1186/1472-6793-12-12.

2.

Cystic fibrosis transmembrane conductance regulator-mRNA delivery: a novel alternative for cystic fibrosis gene therapy.

Bangel-Ruland N, Tomczak K, Fernández Fernández E, Leier G, Leciejewski B, Rudolph C, Rosenecker J, Weber WM.

J Gene Med. 2013 Nov-Dec;15(11-12):414-26. doi: 10.1002/jgm.2748.

PMID:
24123772
3.

Purinergic signaling underlies CFTR control of human airway epithelial cell volume.

Braunstein GM, Zsembery A, Tucker TA, Schwiebert EM.

J Cyst Fibros. 2004 Jun;3(2):99-117.

4.

Endogenous surface expression of ΔF508-CFTR mediates cAMP-stimulated Cl(-) current in CFTR(ΔF508/ΔF508) pig thyroid epithelial cells.

Li Y, Ganta S, Fong P.

Exp Physiol. 2012 Jan;97(1):115-24. doi: 10.1113/expphysiol.2011.060756. Epub 2011 Sep 23.

5.

Effects of cystic fibrosis transmembrane conductance regulator and DeltaF508CFTR on inflammatory response, ER stress, and Ca2+ of airway epithelia.

Hybiske K, Fu Z, Schwarzer C, Tseng J, Do J, Huang N, Machen TE.

Am J Physiol Lung Cell Mol Physiol. 2007 Nov;293(5):L1250-60. Epub 2007 Sep 7.

6.

Extracellular zinc and ATP restore chloride secretion across cystic fibrosis airway epithelia by triggering calcium entry.

Zsembery A, Fortenberry JA, Liang L, Bebok Z, Tucker TA, Boyce AT, Braunstein GM, Welty E, Bell PD, Sorscher EJ, Clancy JP, Schwiebert EM.

J Biol Chem. 2004 Mar 12;279(11):10720-9. Epub 2003 Dec 29.

7.

Characterization of wild-type and deltaF508 cystic fibrosis transmembrane regulator in human respiratory epithelia.

Kreda SM, Mall M, Mengos A, Rochelle L, Yankaskas J, Riordan JR, Boucher RC.

Mol Biol Cell. 2005 May;16(5):2154-67. Epub 2005 Feb 16.

8.

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
9.

Reduced expression of Tis7/IFRD1 protein in murine and human cystic fibrosis airway epithelial cell models homozygous for the F508del-CFTR mutation.

Blanchard E, Marie S, Riffault L, Bonora M, Tabary O, Clement A, Jacquot J.

Biochem Biophys Res Commun. 2011 Aug 5;411(3):471-6. doi: 10.1016/j.bbrc.2011.06.104. Epub 2011 Jun 24.

PMID:
21723850
10.

Partial correction of endogenous DeltaF508 CFTR in human cystic fibrosis airway epithelia by spliceosome-mediated RNA trans-splicing.

Liu X, Jiang Q, Mansfield SG, Puttaraju M, Zhang Y, Zhou W, Cohn JA, Garcia-Blanco MA, Mitchell LG, Engelhardt JF.

Nat Biotechnol. 2002 Jan;20(1):47-52.

PMID:
11753361
11.

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
12.

Correction of delF508-CFTR activity with benzo(c)quinolizinium compounds through facilitation of its processing in cystic fibrosis airway cells.

Dormer RL, Dérand R, McNeilly CM, Mettey Y, Bulteau-Pignoux L, Métayé T, Vierfond JM, Gray MA, Galietta LJ, Morris MR, Pereira MM, Doull IJ, Becq F, McPherson MA.

J Cell Sci. 2001 Nov;114(Pt 22):4073-81.

13.

Expression of CFTR from a ciliated cell-specific promoter is ineffective at correcting nasal potential difference in CF mice.

Ostrowski LE, Yin W, Diggs PS, Rogers TD, O'Neal WK, Grubb BR.

Gene Ther. 2007 Oct;14(20):1492-501. Epub 2007 Jul 19.

PMID:
17637798
14.

The DeltaF508 mutation results in loss of CFTR function and mature protein in native human colon.

Mall M, Kreda SM, Mengos A, Jensen TJ, Hirtz S, Seydewitz HH, Yankaskas J, Kunzelmann K, Riordan JR, Boucher RC.

Gastroenterology. 2004 Jan;126(1):32-41.

PMID:
14699484
15.

Isolation of CF cell lines corrected at DeltaF508-CFTR locus by SFHR-mediated targeting.

Bruscia E, Sangiuolo F, Sinibaldi P, Goncz KK, Novelli G, Gruenert DC.

Gene Ther. 2002 Jun;9(11):683-5.

16.

Improvement of defective cystic fibrosis airway epithelial wound repair after CFTR rescue.

Trinh NT, Bardou O, Privé A, Maillé E, Adam D, Lingée S, Ferraro P, Desrosiers MY, Coraux C, Brochiero E.

Eur Respir J. 2012 Dec;40(6):1390-400. doi: 10.1183/09031936.00221711. Epub 2012 Apr 10.

17.
18.

Comparative processing and function of human and ferret cystic fibrosis transmembrane conductance regulator.

Fisher JT, Liu X, Yan Z, Luo M, Zhang Y, Zhou W, Lee BJ, Song Y, Guo C, Wang Y, Lukacs GL, Engelhardt JF.

J Biol Chem. 2012 Jun 22;287(26):21673-85. doi: 10.1074/jbc.M111.336537. Epub 2012 May 8.

19.

A domain mimic increases DeltaF508 CFTR trafficking and restores cAMP-stimulated anion secretion in cystic fibrosis epithelia.

Clarke LL, Gawenis LR, Hwang TC, Walker NM, Gruis DB, Price EM.

Am J Physiol Cell Physiol. 2004 Jul;287(1):C192-9. Epub 2004 Mar 17.

20.

Defective formation of PKA/CnA-dependent annexin 2-S100A10/CFTR complex in DeltaF508 cystic fibrosis cells.

Borthwick LA, Riemen C, Goddard C, Colledge WH, Mehta A, Gerke V, Muimo R.

Cell Signal. 2008 Jun;20(6):1073-83. doi: 10.1016/j.cellsig.2008.01.021. Epub 2008 Feb 5.

PMID:
18346874

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