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β1-Integrin Accumulates in Cystic Fibrosis Luminal Airway Epithelial Membranes and Decreases Sphingosine, Promoting Bacterial Infections.

Grassmé H, Henry B, Ziobro R, Becker KA, Riethmüller J, Gardner A, Seitz AP, Steinmann J, Lang S, Ward C, Schuchman EH, Caldwell CC, Kamler M, Edwards MJ, Brodlie M, Gulbins E.

Cell Host Microbe. 2017 Jun 14;21(6):707-718.e8. doi: 10.1016/j.chom.2017.05.001. Epub 2017 May 25.


Sodium tanshinone IIA sulfonate stimulated Cl- secretion in mouse trachea.

Chen PX, Zhang YL, Xu JW, Yu MH, Huang JH, Zhao L, Zhou WL.

PLoS One. 2017 May 22;12(5):e0178226. doi: 10.1371/journal.pone.0178226. eCollection 2017.


Effects of Pseudomonas aeruginosa on CFTR chloride secretion and the host immune response.

Stanton BA.

Am J Physiol Cell Physiol. 2017 Apr 1;312(4):C357-C366. doi: 10.1152/ajpcell.00373.2016. Epub 2017 Jan 25. Review.


Effects of treadmill exercise versus Flutter® on respiratory flow and sputum properties in adults with cystic fibrosis: a randomised, controlled, cross-over trial.

Dwyer TJ, Zainuldin R, Daviskas E, Bye PT, Alison JA.

BMC Pulm Med. 2017 Jan 11;17(1):14. doi: 10.1186/s12890-016-0360-8.


Role of IRE1α/XBP-1 in Cystic Fibrosis Airway Inflammation.

Ribeiro CM, Lubamba BA.

Int J Mol Sci. 2017 Jan 9;18(1). pii: E118. doi: 10.3390/ijms18010118. Review.


miR-263a Regulates ENaC to Maintain Osmotic and Intestinal Stem Cell Homeostasis in Drosophila.

Kim K, Hung RJ, Perrimon N.

Dev Cell. 2017 Jan 9;40(1):23-36. doi: 10.1016/j.devcel.2016.11.023. Epub 2016 Dec 22.


Aspergillosis and the role of mucins in cystic fibrosis.

Cowley AC, Thornton DJ, Denning DW, Horsley A.

Pediatr Pulmonol. 2017 Apr;52(4):548-555. doi: 10.1002/ppul.23618. Epub 2016 Nov 21. Review.


Goblet Cell Hyperplasia Requires High Bicarbonate Transport To Support Mucin Release.

Gorrieri G, Scudieri P, Caci E, Schiavon M, Tomati V, Sirci F, Napolitano F, Carrella D, Gianotti A, Musante I, Favia M, Casavola V, Guerra L, Rea F, Ravazzolo R, Di Bernardo D, Galietta LJ.

Sci Rep. 2016 Oct 27;6:36016. doi: 10.1038/srep36016.


Cystic fibrosis transmembrane conductance regulator modulators in cystic fibrosis: current perspectives.

Schmidt BZ, Haaf JB, Leal T, Noel S.

Clin Pharmacol. 2016 Sep 21;8:127-140. eCollection 2016. Review.


CFTR gene transfer with AAV improves early cystic fibrosis pig phenotypes.

Steines B, Dickey DD, Bergen J, Excoffon KJ, Weinstein JR, Li X, Yan Z, Abou Alaiwa MH, Shah VS, Bouzek DC, Powers LS, Gansemer ND, Ostedgaard LS, Engelhardt JF, Stoltz DA, Welsh MJ, Sinn PL, Schaffer DV, Zabner J.

JCI Insight. 2016 Sep 8;1(14):e88728.


An in vitro model of murine middle ear epithelium.

Mulay A, Akram KM, Williams D, Armes H, Russell C, Hood D, Armstrong S, Stewart JP, Brown SD, Bingle L, Bingle CD.

Dis Model Mech. 2016 Nov 1;9(11):1405-1417. Epub 2016 Sep 22.


The Mucus Barrier to Inhaled Gene Therapy.

Duncan GA, Jung J, Hanes J, Suk JS.

Mol Ther. 2016 Dec;24(12):2043-2053. doi: 10.1038/mt.2016.182. Epub 2016 Sep 20. Review.


Repurposing tromethamine as inhaled therapy to treat CF airway disease.

Abou Alaiwa MH, Launspach JL, Sheets KA, Rivera JA, Gansemer ND, Taft PJ, Thorne PS, Welsh MJ, Stoltz DA, Zabner J.

JCI Insight. 2016 Jun 2;1(8). pii: e87535.


Epithelial Anion Transport as Modulator of Chemokine Signaling.

Schnúr A, Hegyi P, Rousseau S, Lukacs GL, Veit G.

Mediators Inflamm. 2016;2016:7596531. doi: 10.1155/2016/7596531. Epub 2016 Jun 12. Review.


Bacterial evolution in PCD and CF patients follows the same mutational steps.

Sommer LM, Alanin MC, Marvig RL, Nielsen KG, Høiby N, von Buchwald C, Molin S, Johansen HK.

Sci Rep. 2016 Jun 28;6:28732. doi: 10.1038/srep28732.


Current and future treatment options for cystic fibrosis lung disease: latest evidence and clinical implications.

Edmondson C, Davies JC.

Ther Adv Chronic Dis. 2016 May;7(3):170-83. doi: 10.1177/2040622316641352. Epub 2016 May 1. Review.


A proposed integrated approach for the preclinical evaluation of phage therapy in Pseudomonas infections.

Danis-Wlodarczyk K, Vandenheuvel D, Jang HB, Briers Y, Olszak T, Arabski M, Wasik S, Drabik M, Higgins G, Tyrrell J, Harvey BJ, Noben JP, Lavigne R, Drulis-Kawa Z.

Sci Rep. 2016 Jun 15;6:28115. doi: 10.1038/srep28115.


Chelation of Membrane-Bound Cations by Extracellular DNA Activates the Type VI Secretion System in Pseudomonas aeruginosa.

Wilton M, Wong MJ, Tang L, Liang X, Moore R, Parkins MD, Lewenza S, Dong TG.

Infect Immun. 2016 Jul 21;84(8):2355-61. doi: 10.1128/IAI.00233-16. Print 2016 Aug.


Barriers to inhaled gene therapy of obstructive lung diseases: A review.

Kim N, Duncan GA, Hanes J, Suk JS.

J Control Release. 2016 Oct 28;240:465-488. doi: 10.1016/j.jconrel.2016.05.031. Epub 2016 May 16.


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