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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.


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.


Na+/H+ exchanger regulatory factor isoform 1 overexpression modulates cystic fibrosis transmembrane conductance regulator (CFTR) expression and activity in human airway 16HBE14o- cells and rescues DeltaF508 CFTR functional expression in cystic fibrosis cells.

Guerra L, Fanelli T, Favia M, Riccardi SM, Busco G, Cardone RA, Carrabino S, Weinman EJ, Reshkin SJ, Conese M, Casavola V.

J Biol Chem. 2005 Dec 9;280(49):40925-33. Epub 2005 Oct 3.


Decreased expression of the CFTR protein in remodeled human nasal epithelium from non-cystic fibrosis patients.

Brezillon S, Dupuit F, Hinnrasky J, Marchand V, Kälin N, Tümmler B, Puchelle E.

Lab Invest. 1995 Feb;72(2):191-200.


The role of the C terminus and Na+/H+ exchanger regulatory factor in the functional expression of cystic fibrosis transmembrane conductance regulator in nonpolarized cells and epithelia.

Benharouga M, Sharma M, So J, Haardt M, Drzymala L, Popov M, Schwapach B, Grinstein S, Du K, Lukacs GL.

J Biol Chem. 2003 Jun 13;278(24):22079-89. Epub 2003 Mar 21.


CFTR and differentiation markers expression in non-CF and delta F 508 homozygous CF nasal epithelium.

Dupuit F, Kälin N, Brézillon S, Hinnrasky J, Tümmler B, Puchelle E.

J Clin Invest. 1995 Sep;96(3):1601-11.


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.


DeltaF508 CFTR protein expression in tissues from patients with cystic fibrosis.

Kälin N, Claass A, Sommer M, Puchelle E, Tümmler B.

J Clin Invest. 1999 May 15;103(10):1379-89.


CFTR chloride channel drug discovery--inhibitors as antidiarrheals and activators for therapy of cystic fibrosis.

Verkman AS, Lukacs GL, Galietta LJ.

Curr Pharm Des. 2006;12(18):2235-47. Review.


Localisation of wild-type and DeltaF508-CFTR in nasal epithelial cells.

Dormer RL, McNeilly CM, Morris MR, Pereira MM, Doull IJ, Becq F, Mettey Y, Vierfond JM, McPherson MA.

Pflugers Arch. 2001;443 Suppl 1:S117-20. Epub 2001 Jul 13.


Rescue of DeltaF508-CFTR (cystic fibrosis transmembrane conductance regulator) by curcumin: involvement of the keratin 18 network.

Lipecka J, Norez C, Bensalem N, Baudouin-Legros M, Planelles G, Becq F, Edelman A, Davezac N.

J Pharmacol Exp Ther. 2006 May;317(2):500-5. Epub 2006 Jan 19.


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.


Mild processing defect of porcine DeltaF508-CFTR suggests that DeltaF508 pigs may not develop cystic fibrosis disease.

Liu Y, Wang Y, Jiang Y, Zhu N, Liang H, Xu L, Feng X, Yang H, Ma T.

Biochem Biophys Res Commun. 2008 Aug 15;373(1):113-8. doi: 10.1016/j.bbrc.2008.06.009. Epub 2008 Jun 12.


Repeat administration of DNA/liposomes to the nasal epithelium of patients with cystic fibrosis.

Hyde SC, Southern KW, Gileadi U, Fitzjohn EM, Mofford KA, Waddell BE, Gooi HC, Goddard CA, Hannavy K, Smyth SE, Egan JJ, Sorgi FL, Huang L, Cuthbert AW, Evans MJ, Colledge WH, Higgins CF, Webb AK, Gill DR.

Gene Ther. 2000 Jul;7(13):1156-65.


Expression of cystic fibrosis transmembrane conductance regulator in liver tissue from patients with cystic fibrosis.

Kinnman N, Lindblad A, Housset C, Buentke E, Scheynius A, Strandvik B, Hultcrantz R.

Hepatology. 2000 Aug;32(2):334-40.


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.


Glucose homeostasis and genotype-phenotype interplay in cystic fibrosis patients with CFTR gene deltaF508 mutation.

Preumont V, Hermans MP, Lebecque P, Buysschaert M.

Diabetes Care. 2007 May;30(5):1187-92. Epub 2007 Mar 2.


The mechanism of cystic fibrosis transmembrane conductance regulator transcriptional repression during the unfolded protein response.

Bartoszewski R, Rab A, Twitty G, Stevenson L, Fortenberry J, Piotrowski A, Dumanski JP, Bebok Z.

J Biol Chem. 2008 May 2;283(18):12154-65. doi: 10.1074/jbc.M707610200. Epub 2008 Mar 4.

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