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Items: 1 to 20 of 49

1.

Quantitative methods in the study of pulmonary pathology.

Dunnill MS.

Thorax. 1962 Dec;17(4):320-28. No abstract available.

2.

A pharmacologic approach to acquired cystic fibrosis transmembrane conductance regulator dysfunction in smoking related lung disease.

Sloane PA, Shastry S, Wilhelm A, Courville C, Tang LP, Backer K, Levin E, Raju SV, Li Y, Mazur M, Byan-Parker S, Grizzle W, Sorscher EJ, Dransfield MT, Rowe SM.

PLoS One. 2012;7(6):e39809. doi: 10.1371/journal.pone.0039809. Epub 2012 Jun 29.

3.

The CFTR and ENaC debate: how important is ENaC in CF lung disease?

Collawn JF, Lazrak A, Bebok Z, Matalon S.

Am J Physiol Lung Cell Mol Physiol. 2012 Jun 1;302(11):L1141-6. doi: 10.1152/ajplung.00036.2012. Epub 2012 Apr 6.

4.

Genetically determined heterogeneity of lung disease in a mouse model of airway mucus obstruction.

Livraghi-Butrico A, Grubb BR, Kelly EJ, Wilkinson KJ, Yang H, Geiser M, Randell SH, Boucher RC, O'Neal WK.

Physiol Genomics. 2012 Apr 15;44(8):470-84. doi: 10.1152/physiolgenomics.00185.2011. Epub 2012 Mar 6.

5.

A CFTR potentiator in patients with cystic fibrosis and the G551D mutation.

Ramsey BW, Davies J, McElvaney NG, Tullis E, Bell SC, Dřevínek P, Griese M, McKone EF, Wainwright CE, Konstan MW, Moss R, Ratjen F, Sermet-Gaudelus I, Rowe SM, Dong Q, Rodriguez S, Yen K, Ordoñez C, Elborn JS; VX08-770-102 Study Group.

N Engl J Med. 2011 Nov 3;365(18):1663-72. doi: 10.1056/NEJMoa1105185.

6.

Transgenic hCFTR expression fails to correct β-ENaC mouse lung disease.

Grubb BR, O'Neal WK, Ostrowski LE, Kreda SM, Button B, Boucher RC.

Am J Physiol Lung Cell Mol Physiol. 2012 Jan 15;302(2):L238-47. doi: 10.1152/ajplung.00083.2011. Epub 2011 Oct 14.

7.

Cigarette smoke exposure induces CFTR internalization and insolubility, leading to airway surface liquid dehydration.

Clunes LA, Davies CM, Coakley RD, Aleksandrov AA, Henderson AG, Zeman KL, Worthington EN, Gentzsch M, Kreda SM, Cholon D, Bennett WD, Riordan JR, Boucher RC, Tarran R.

FASEB J. 2012 Feb;26(2):533-45. doi: 10.1096/fj.11-192377. Epub 2011 Oct 11.

8.

The K+ channel opener 1-EBIO potentiates residual function of mutant CFTR in rectal biopsies from cystic fibrosis patients.

Roth EK, Hirtz S, Duerr J, Wenning D, Eichler I, Seydewitz HH, Amaral MD, Mall MA.

PLoS One. 2011;6(8):e24445. doi: 10.1371/journal.pone.0024445. Epub 2011 Aug 31.

9.

The ENaC-overexpressing mouse as a model of cystic fibrosis lung disease.

Zhou Z, Duerr J, Johannesson B, Schubert SC, Treis D, Harm M, Graeber SY, Dalpke A, Schultz C, Mall MA.

J Cyst Fibros. 2011 Jun;10 Suppl 2:S172-82. doi: 10.1016/S1569-1993(11)60021-0. Review.

10.

Pharmacological therapy for cystic fibrosis: from bench to bedside.

Becq F, Mall MA, Sheppard DN, Conese M, Zegarra-Moran O.

J Cyst Fibros. 2011 Jun;10 Suppl 2:S129-45. doi: 10.1016/S1569-1993(11)60018-0. Review.

11.

Genetics of complex airway disease.

Cookson WO, Moffatt MF.

Proc Am Thorac Soc. 2011 May;8(2):149-53. doi: 10.1513/pats.201101-003MS. Review.

12.

In vivo monitoring of cystic fibrosis-like lung disease in mice by volumetric computed tomography.

Wielpütz MO, Eichinger M, Zhou Z, Leotta K, Hirtz S, Bartling SH, Semmler W, Kauczor HU, Puderbach M, Mall MA.

Eur Respir J. 2011 Nov;38(5):1060-70. doi: 10.1183/09031936.00149810. Epub 2011 Apr 8.

13.

Critical role of CFTR-dependent lipid rafts in cigarette smoke-induced lung epithelial injury.

Bodas M, Min T, Vij N.

Am J Physiol Lung Cell Mol Physiol. 2011 Jun;300(6):L811-20. doi: 10.1152/ajplung.00408.2010. Epub 2011 Mar 4.

14.

Critical modifier role of membrane-cystic fibrosis transmembrane conductance regulator-dependent ceramide signaling in lung injury and emphysema.

Bodas M, Min T, Mazur S, Vij N.

J Immunol. 2011 Jan 1;186(1):602-13. doi: 10.4049/jimmunol.1002850. Epub 2010 Dec 6.

15.

Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation.

Accurso FJ, Rowe SM, Clancy JP, Boyle MP, Dunitz JM, Durie PR, Sagel SD, Hornick DB, Konstan MW, Donaldson SH, Moss RB, Pilewski JM, Rubenstein RC, Uluer AZ, Aitken ML, Freedman SD, Rose LM, Mayer-Hamblett N, Dong Q, Zha J, Stone AJ, Olson ER, Ordoñez CL, Campbell PW, Ashlock MA, Ramsey BW.

N Engl J Med. 2010 Nov 18;363(21):1991-2003. doi: 10.1056/NEJMoa0909825.

16.

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.

17.

Airway surface liquid volume regulation determines different airway phenotypes in liddle compared with betaENaC-overexpressing mice.

Mall MA, Button B, Johannesson B, Zhou Z, Livraghi A, Caldwell RA, Schubert SC, Schultz C, O'Neal WK, Pradervand S, Hummler E, Rossier BC, Grubb BR, Boucher RC.

J Biol Chem. 2010 Aug 27;285(35):26945-55. doi: 10.1074/jbc.M110.151803. Epub 2010 Jun 21.

18.

Allergic airway inflammation induces a pro-secretory epithelial ion transport phenotype in mice.

Anagnostopoulou P, Dai L, Schatterny J, Hirtz S, Duerr J, Mall MA.

Eur Respir J. 2010 Dec;36(6):1436-47. doi: 10.1183/09031936.00181209. Epub 2010 Apr 22. Erratum in: Eur Respir J. 2011 Mar;37(3):727.

19.

Use of a new-generation reverse tetracycline transactivator system for quantitative control of conditional gene expression in the murine lung.

Duerr J, Gruner M, Schubert SC, Haberkorn U, Bujard H, Mall MA.

Am J Respir Cell Mol Biol. 2011 Feb;44(2):244-54. doi: 10.1165/rcmb.2009-0115OC. Epub 2010 Apr 15.

PMID:
20395635
20.

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.

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