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Failure of cAMP agonists to activate rescued deltaF508 CFTR in CFBE41o- airway epithelial monolayers.

Bebok Z, Collawn JF, Wakefield J, Parker W, Li Y, Varga K, Sorscher EJ, Clancy JP.

J Physiol. 2005 Dec 1;569(Pt 2):601-15.


DeltaF508 CFTR processing correction and activity in polarized airway and non-airway cell monolayers.

Rowe SM, Pyle LC, Jurkevante A, Varga K, Collawn J, Sloane PA, Woodworth B, Mazur M, Fulton J, Fan L, Li Y, Fortenberry J, Sorscher EJ, Clancy JP.

Pulm Pharmacol Ther. 2010 Aug;23(4):268-78. doi: 10.1016/j.pupt.2010.02.001.


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.


Enhanced cell-surface stability of rescued DeltaF508 cystic fibrosis transmembrane conductance regulator (CFTR) by pharmacological chaperones.

Varga K, Goldstein RF, Jurkuvenaite A, Chen L, Matalon S, Sorscher EJ, Bebok Z, Collawn JF.

Biochem J. 2008 Mar 15;410(3):555-64.


Activation of the cystic fibrosis transmembrane conductance regulator by the flavonoid quercetin: potential use as a biomarker of ΔF508 cystic fibrosis transmembrane conductance regulator rescue.

Pyle LC, Fulton JC, Sloane PA, Backer K, Mazur M, Prasain J, Barnes S, Clancy JP, Rowe SM.

Am J Respir Cell Mol Biol. 2010 Nov;43(5):607-16. doi: 10.1165/rcmb.2009-0281OC.


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.


Targeting autophagy as a novel strategy for facilitating the therapeutic action of potentiators on ΔF508 cystic fibrosis transmembrane conductance regulator.

Luciani A, Villella VR, Esposito S, Gavina M, Russo I, Silano M, Guido S, Pettoello-Mantovani M, Carnuccio R, Scholte B, De Matteis A, Maiuri MC, Raia V, Luini A, Kroemer G, Maiuri L.

Autophagy. 2012 Nov;8(11):1657-72. doi: 10.4161/auto.21483.


Trafficking of GFP-tagged DeltaF508-CFTR to the plasma membrane in a polarized epithelial cell line.

Loffing-Cueni D, Loffing J, Shaw C, Taplin AM, Govindan M, Stanton CR, Stanton BA.

Am J Physiol Cell Physiol. 2001 Dec;281(6):C1889-97.


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.


CFTR regulation in human airway epithelial cells requires integrity of the actin cytoskeleton and compartmentalized cAMP and PKA activity.

Monterisi S, Favia M, Guerra L, Cardone RA, Marzulli D, Reshkin SJ, Casavola V, Zaccolo M.

J Cell Sci. 2012 Mar 1;125(Pt 5):1106-17. doi: 10.1242/jcs.089086.


Expression and function of cystic fibrosis transmembrane conductance regulator in rat intrapulmonary arteries.

Robert R, Savineau JP, Norez C, Becq F, Guibert C.

Eur Respir J. 2007 Nov;30(5):857-64.


cAMP and genistein stimulate HCO3- conductance through CFTR in human airway epithelia.

Illek B, Yankaskas JR, Machen TE.

Am J Physiol. 1997 Apr;272(4 Pt 1):L752-61.


Tgf-β1 inhibits Cftr biogenesis and prevents functional rescue of ΔF508-Cftr in primary differentiated human bronchial epithelial cells.

Snodgrass SM, Cihil KM, Cornuet PK, Myerburg MM, Swiatecka-Urban A.

PLoS One. 2013 May 9;8(5):e63167. doi: 10.1371/journal.pone.0063167.

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