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Left ventricular and aortic dysfunction in cystic fibrosis mice.

Sellers ZM, Kovacs A, Weinheimer CJ, Best PM.

J Cyst Fibros. 2013 Sep;12(5):517-24. doi: 10.1016/j.jcf.2012.11.012. Epub 2012 Dec 24.


The impact of Cystic Fibrosis Transmembrane Regulator Disruption on cardiac function and stress response.

Jiang K, Jiao S, Vitko M, Darrah R, Flask CA, Hodges CA, Yu X.

J Cyst Fibros. 2016 Jan;15(1):34-42. doi: 10.1016/j.jcf.2015.06.003. Epub 2015 Jun 25.


Strain rate echocardiography uncovers subclinical left ventricular dysfunction in cystic fibrosis.

Sellers ZM, McGlocklin L, Brasch A.

J Cyst Fibros. 2015 Sep;14(5):654-60. doi: 10.1016/j.jcf.2015.03.010. Epub 2015 Apr 9.


Regulation of cystic fibrosis transmembrane conductance regulator by microRNA-145, -223, and -494 is altered in ΔF508 cystic fibrosis airway epithelium.

Oglesby IK, Chotirmall SH, McElvaney NG, Greene CM.

J Immunol. 2013 Apr 1;190(7):3354-62. doi: 10.4049/jimmunol.1202960. Epub 2013 Feb 22.


Reduced blood pressure of CFTR-F508del carriers correlates with diminished arterial reactivity rather than circulating blood volume in mice.

Peotta VA, Bhandary P, Ogu U, Volk KA, Roghair RD.

PLoS One. 2014 May 6;9(5):e96756. doi: 10.1371/journal.pone.0096756. eCollection 2014.


The F508del mutation in cystic fibrosis transmembrane conductance regulator gene impacts bone formation.

Le Henaff C, Gimenez A, Haÿ E, Marty C, Marie P, Jacquot J.

Am J Pathol. 2012 May;180(5):2068-75. doi: 10.1016/j.ajpath.2012.01.039. Epub 2012 Mar 23.


CFTR: cystic fibrosis and beyond.

Mall MA, Hartl D.

Eur Respir J. 2014 Oct;44(4):1042-54. doi: 10.1183/09031936.00228013. Epub 2014 Jun 12.


Cystic fibrosis transmembrane conductance regulator does not affect neutrophil migration across cystic fibrosis airway epithelial monolayers.

Pizurki L, Morris MA, Chanson M, Solomon M, Pavirani A, Bouchardy I, Suter S.

Am J Pathol. 2000 Apr;156(4):1407-16. Erratum in: Am J Pathol 2000 Oct;157(4):1413.


Renal proximal tubule function is preserved in Cftr(tm2cam) deltaF508 cystic fibrosis mice.

Kibble JD, Balloch KJ, Neal AM, Hill C, White S, Robson L, Green R, Taylor CJ.

J Physiol. 2001 Apr 15;532(Pt 2):449-57.


Rescue of DeltaF508-CFTR trafficking and gating in human cystic fibrosis airway primary cultures by small molecules.

Van Goor F, Straley KS, Cao D, González J, Hadida S, Hazlewood A, Joubran J, Knapp T, Makings LR, Miller M, Neuberger T, Olson E, Panchenko V, Rader J, Singh A, Stack JH, Tung R, Grootenhuis PD, Negulescu P.

Am J Physiol Lung Cell Mol Physiol. 2006 Jun;290(6):L1117-30. Epub 2006 Jan 27.


Genetic deletion of keratin 8 corrects the altered bone formation and osteopenia in a mouse model of cystic fibrosis.

Le Henaff C, Faria Da Cunha M, Hatton A, Tondelier D, Marty C, Collet C, Zarka M, Geoffroy V, Zatloukal K, Laplantine E, Edelman A, Sermet-Gaudelus I, Marie PJ.

Hum Mol Genet. 2016 Apr 1;25(7):1281-93. doi: 10.1093/hmg/ddw009. Epub 2016 Jan 13.


DeltaF508-CFTR causes constitutive NF-kappaB activation through an ER-overload response in cystic fibrosis lungs.

Knorre A, Wagner M, Schaefer HE, Colledge WH, Pahl HL.

Biol Chem. 2002 Feb;383(2):271-82.


Cystic fibrosis transmembrane conductance regulator in human muscle: Dysfunction causes abnormal metabolic recovery in exercise.

Lamhonwah AM, Bear CE, Huan LJ, Kim Chiaw P, Ackerley CA, Tein I.

Ann Neurol. 2010 Jun;67(6):802-8. doi: 10.1002/ana.21982.


A mutation in the cystic fibrosis transmembrane conductance regulator gene associated with elevated sweat chloride concentrations in the absence of cystic fibrosis.

Mickle JE, Macek M Jr, Fulmer-Smentek SB, Egan MM, Schwiebert E, Guggino W, Moss R, Cutting GR.

Hum Mol Genet. 1998 Apr;7(4):729-35.


Aggregates of mutant CFTR fragments in airway epithelial cells of CF lungs: new pathologic observations.

Du K, Karp PH, Ackerley C, Zabner J, Keshavjee S, Cutz E, Yeger H.

J Cyst Fibros. 2015 Mar;14(2):182-93. doi: 10.1016/j.jcf.2014.09.012. Epub 2014 Oct 28.


Cystic fibrosis transmembrane conductance regulator channel dysfunction in non-cystic fibrosis bronchiectasis.

Bienvenu T, Sermet-Gaudelus I, Burgel PR, Hubert D, Crestani B, Bassinet L, Dusser D, Fajac I.

Am J Respir Crit Care Med. 2010 May 15;181(10):1078-84. doi: 10.1164/rccm.200909-1434OC. Epub 2010 Feb 18.


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