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Regulation of endogenous ENaC functional expression by CFTR and ΔF508-CFTR in airway epithelial cells.

Rubenstein RC, Lockwood SR, Lide E, Bauer R, Suaud L, Grumbach Y.

Am J Physiol Lung Cell Mol Physiol. 2011 Jan;300(1):L88-L101. doi: 10.1152/ajplung.00142.2010. Epub 2010 Oct 8.


Cystic fibrosis transmembrane conductance regulator protein repair as a therapeutic strategy in cystic fibrosis.

Sloane PA, Rowe SM.

Curr Opin Pulm Med. 2010 Nov;16(6):591-7. doi: 10.1097/MCP.0b013e32833f1d00. Review.


Correction of the Delta phe508 cystic fibrosis transmembrane conductance regulator trafficking defect by the bioavailable compound glafenine.

Robert R, Carlile GW, Liao J, Balghi H, Lesimple P, Liu N, Kus B, Rotin D, Wilke M, de Jonge HR, Scholte BJ, Thomas DY, Hanrahan JW.

Mol Pharmacol. 2010 Jun;77(6):922-30. doi: 10.1124/mol.109.062679. Epub 2010 Mar 3.


Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770.

Van Goor F, Hadida S, Grootenhuis PD, Burton B, Cao D, Neuberger T, Turnbull A, Singh A, Joubran J, Hazlewood A, Zhou J, McCartney J, Arumugam V, Decker C, Yang J, Young C, Olson ER, Wine JJ, Frizzell RA, Ashlock M, Negulescu P.

Proc Natl Acad Sci U S A. 2009 Nov 3;106(44):18825-30. doi: 10.1073/pnas.0904709106. Epub 2009 Oct 21.


Rescue of DeltaF508-CFTR by the SGK1/Nedd4-2 signaling pathway.

Caohuy H, Jozwik C, Pollard HB.

J Biol Chem. 2009 Sep 11;284(37):25241-53. doi: 10.1074/jbc.M109.035345. Epub 2009 Jul 17.


Discordant effects of corticosteroids and expression of subunits on ENaC activity.

Husted RF, Volk KA, Sigmund RD, Stokes JB.

Am J Physiol Renal Physiol. 2007 Sep;293(3):F813-20. Epub 2007 Jul 3.


Nedd4-2 catalyzes ubiquitination and degradation of cell surface ENaC.

Zhou R, Patel SV, Snyder PM.

J Biol Chem. 2007 Jul 13;282(28):20207-12. Epub 2007 May 14.


Chloride-dependent secretion of alveolar wall liquid determined by optical-sectioning microscopy.

Lindert J, Perlman CE, Parthasarathi K, Bhattacharya J.

Am J Respir Cell Mol Biol. 2007 Jun;36(6):688-96. Epub 2007 Feb 8.


WNK1 and WNK4 modulate CFTR activity.

Yang CL, Liu X, Paliege A, Zhu X, Bachmann S, Dawson DC, Ellison DH.

Biochem Biophys Res Commun. 2007 Feb 16;353(3):535-40. Epub 2006 Dec 15.


New insights into cystic fibrosis: molecular switches that regulate CFTR.

Guggino WB, Stanton BA.

Nat Rev Mol Cell Biol. 2006 Jun;7(6):426-36.


Ion and fluid transport properties of small airways in cystic fibrosis.

Blouquit S, Regnier A, Dannhoffer L, Fermanian C, Naline E, Boucher R, Chinet T.

Am J Respir Crit Care Med. 2006 Aug 1;174(3):299-305. Epub 2006 Apr 27.


Influence of IFN-gamma on gene expression in normal human bronchial epithelial cells: modulation of IFN-gamma effects by dexamethasone.

Pawliczak R, Logun C, Madara P, Barb J, Suffredini AF, Munson PJ, Danner RL, Shelhamer JH.

Physiol Genomics. 2005 Sep 21;23(1):28-45. Epub 2005 Jun 28.


The CFTR gene and regulation of its expression.

McCarthy VA, Harris A.

Pediatr Pulmonol. 2005 Jul;40(1):1-8. Review.


The cystic fibrosis transmembrane conductance regulator: an intriguing protein with pleiotropic functions.

Vankeerberghen A, Cuppens H, Cassiman JJ.

J Cyst Fibros. 2002 Mar;1(1):13-29. Review.


Low doses of dexamethasone constantly delivered by autologous erythrocytes slow the progression of lung disease in cystic fibrosis patients.

Rossi L, Castro M, D'Orio F, Damonte G, Serafini S, Bigi L, Panzani I, Novelli G, Dallapiccola B, Panunzi S, Di Carlo P, Bella S, Magnani M.

Blood Cells Mol Dis. 2004 Jul-Aug;33(1):57-63.


Pathophysiology and management of pulmonary infections in cystic fibrosis.

Gibson RL, Burns JL, Ramsey BW.

Am J Respir Crit Care Med. 2003 Oct 15;168(8):918-51. Review.


The role of regulated CFTR trafficking in epithelial secretion.

Bertrand CA, Frizzell RA.

Am J Physiol Cell Physiol. 2003 Jul;285(1):C1-18. Review.


Cystic fibrosis transmembrane conductance regulator-dependent up-regulation of Kir1.1 (ROMK) renal K+ channels by the epithelial sodium channel.

Konstas AA, Koch JP, Tucker SJ, Korbmacher C.

J Biol Chem. 2002 Jul 12;277(28):25377-84. Epub 2002 May 6.


Glucocorticoid-stimulated lung epithelial Na(+) transport is associated with regulated ENaC and sgk1 expression.

Itani OA, Auerbach SD, Husted RF, Volk KA, Ageloff S, Knepper MA, Stokes JB, Thomas CP.

Am J Physiol Lung Cell Mol Physiol. 2002 Apr;282(4):L631-41.


Temporal regulation of CFTR expression during ovine lung development: implications for CF gene therapy.

Broackes-Carter FC, Mouchel N, Gill D, Hyde S, Bassett J, Harris A.

Hum Mol Genet. 2002 Jan 15;11(2):125-31.

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