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17beta-Estradiol inhibits Ca2+-dependent homeostasis of airway surface liquid volume in human cystic fibrosis airway epithelia.

Coakley RD, Sun H, Clunes LA, Rasmussen JE, Stackhouse JR, Okada SF, Fricks I, Young SL, Tarran R.

J Clin Invest. 2008 Dec;118(12):4025-35. doi: 10.1172/JCI33893. Epub 2008 Nov 20.


Disruption of the CFTR gene produces a model of cystic fibrosis in newborn pigs.

Rogers CS, Stoltz DA, Meyerholz DK, Ostedgaard LS, Rokhlina T, Taft PJ, Rogan MP, Pezzulo AA, Karp PH, Itani OA, Kabel AC, Wohlford-Lenane CL, Davis GJ, Hanfland RA, Smith TL, Samuel M, Wax D, Murphy CN, Rieke A, Whitworth K, Uc A, Starner TD, Brogden KA, Shilyansky J, McCray PB Jr, Zabner J, Prather RS, Welsh MJ.

Science. 2008 Sep 26;321(5897):1837-41. doi: 10.1126/science.1163600.


The porcine lung as a potential model for cystic fibrosis.

Rogers CS, Abraham WM, Brogden KA, Engelhardt JF, Fisher JT, McCray PB Jr, McLennan G, Meyerholz DK, Namati E, Ostedgaard LS, Prather RS, Sabater JR, Stoltz DA, Zabner J, Welsh MJ.

Am J Physiol Lung Cell Mol Physiol. 2008 Aug;295(2):L240-63. doi: 10.1152/ajplung.90203.2008. Epub 2008 May 16. Review.


Prostasin expression is regulated by airway surface liquid volume and is increased in cystic fibrosis.

Myerburg MM, McKenna EE, Luke CJ, Frizzell RA, Kleyman TR, Pilewski JM.

Am J Physiol Lung Cell Mol Physiol. 2008 May;294(5):L932-41. doi: 10.1152/ajplung.00437.2007. Epub 2008 Feb 29.


Enhanced gene expression conferred by stepwise modification of a nonprimate lentiviral vector.

Sinn PL, Goreham-Voss JD, Arias AC, Hickey MA, Maury W, Chikkanna-Gowda CP, McCray PB Jr.

Hum Gene Ther. 2007 Dec;18(12):1244-52.


Repeated aerosolized AAV-CFTR for treatment of cystic fibrosis: a randomized placebo-controlled phase 2B trial.

Moss RB, Milla C, Colombo J, Accurso F, Zeitlin PL, Clancy JP, Spencer LT, Pilewski J, Waltz DA, Dorkin HL, Ferkol T, Pian M, Ramsey B, Carter BJ, Martin DB, Heald AE.

Hum Gene Ther. 2007 Aug;18(8):726-32.


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.


Acinar origin of CFTR-dependent airway submucosal gland fluid secretion.

Wu JV, Krouse ME, Wine JJ.

Am J Physiol Lung Cell Mol Physiol. 2007 Jan;292(1):L304-11. Epub 2006 Sep 22.


Effective mucus clearance is essential for respiratory health.

Randell SH, Boucher RC; University of North Carolina Virtual Lung Group.

Am J Respir Cell Mol Biol. 2006 Jul;35(1):20-8. Epub 2006 Mar 9. Review. No abstract available.


Mucus clearance and lung function in cystic fibrosis with hypertonic saline.

Donaldson SH, Bennett WD, Zeman KL, Knowles MR, Tarran R, Boucher RC.

N Engl J Med. 2006 Jan 19;354(3):241-50.


Influenza M2 envelope protein augments avian influenza hemagglutinin pseudotyping of lentiviral vectors.

McKay T, Patel M, Pickles RJ, Johnson LG, Olsen JC.

Gene Ther. 2006 Apr;13(8):715-24.


SERCA pump inhibitors do not correct biosynthetic arrest of deltaF508 CFTR in cystic fibrosis.

Grubb BR, Gabriel SE, Mengos A, Gentzsch M, Randell SH, Van Heeckeren AM, Knowles MR, Drumm ML, Riordan JR, Boucher RC.

Am J Respir Cell Mol Biol. 2006 Mar;34(3):355-63. Epub 2005 Nov 11.


Persistent gene expression in mouse nasal epithelia following feline immunodeficiency virus-based vector gene transfer.

Sinn PL, Burnight ER, Hickey MA, Blissard GW, McCray PB Jr.

J Virol. 2005 Oct;79(20):12818-27.


Normal and cystic fibrosis airway surface liquid homeostasis. The effects of phasic shear stress and viral infections.

Tarran R, Button B, Picher M, Paradiso AM, Ribeiro CM, Lazarowski ER, Zhang L, Collins PL, Pickles RJ, Fredberg JJ, Boucher RC.

J Biol Chem. 2005 Oct 21;280(42):35751-9. Epub 2005 Aug 8.


Gene transfer of CFTR to airway epithelia: low levels of expression are sufficient to correct Cl- transport and overexpression can generate basolateral CFTR.

Farmen SL, Karp PH, Ng P, Palmer DJ, Koehler DR, Hu J, Beaudet AL, Zabner J, Welsh MJ.

Am J Physiol Lung Cell Mol Physiol. 2005 Dec;289(6):L1123-30. Epub 2005 Aug 5.


Towards a gene therapy for cystic fibrosis lung disease.

Pickles RJ.

Expert Opin Investig Drugs. 1997 Oct;6(10):1459-63.


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.


Infection of ciliated cells by human parainfluenza virus type 3 in an in vitro model of human airway epithelium.

Zhang L, Bukreyev A, Thompson CI, Watson B, Peeples ME, Collins PL, Pickles RJ.

J Virol. 2005 Jan;79(2):1113-24.

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