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Dysregulated proinflammatory and fibrogenic phenotype of fibroblasts in cystic fibrosis.

Huaux F, Noel S, Dhooghe B, Panin N, Lo Re S, Lison D, Wallemacq P, Marbaix E, Scholte BJ, Lebecque P, Leal T.

PLoS One. 2013 May 29;8(5):e64341. doi: 10.1371/journal.pone.0064341. Print 2013.


Correction of chloride transport and mislocalization of CFTR protein by vardenafil in the gastrointestinal tract of cystic fibrosis mice.

Dhooghe B, Noël S, Bouzin C, Behets-Wydemans G, Leal T.

PLoS One. 2013 Oct 24;8(10):e77314. doi: 10.1371/journal.pone.0077314. eCollection 2013.


Immunomodulatory activity of vardenafil on induced lung inflammation in cystic fibrosis mice.

Lubamba B, Huaux F, Lebacq J, Marbaix E, Dhooghe B, Panin N, Wallemacq P, Leal T.

J Cyst Fibros. 2012 Jul;11(4):266-73. doi: 10.1016/j.jcf.2012.03.003. Epub 2012 Apr 12.


Azithromycin reduces exaggerated cytokine production by M1 alveolar macrophages in cystic fibrosis.

Meyer M, Huaux F, Gavilanes X, van den Brûle S, Lebecque P, Lo Re S, Lison D, Scholte B, Wallemacq P, Leal T.

Am J Respir Cell Mol Biol. 2009 Nov;41(5):590-602. doi: 10.1165/rcmb.2008-0155OC. Epub 2009 Feb 24.


Vardenafil reduces macrophage pro-inflammatory overresponses in cystic fibrosis through PDE5- and CFTR-dependent mechanisms.

Noel S, Panin N, Beka M, Dhooghe B, Huaux F, Leal T.

Clin Sci (Lond). 2017 Jun 1;131(11):1107-1121. doi: 10.1042/CS20160749. Epub 2017 Feb 14.


Strain-dependent pulmonary gene expression profiles of a cystic fibrosis mouse model.

Haston CK, Cory S, Lafontaine L, Dorion G, Hallett MT.

Physiol Genomics. 2006 Apr 13;25(2):336-45.


Inflammation in cystic fibrosis airways: relationship to increased bacterial adherence.

Scheid P, Kempster L, Griesenbach U, Davies JC, Dewar A, Weber PP, Colledge WH, Evans MJ, Geddes DM, Alton EW.

Eur Respir J. 2001 Jan;17(1):27-35.


Delivery of CFTR by adenoviral vector to cystic fibrosis mouse lung in a model of chronic Pseudomonas aeruginosa lung infection.

Van Heeckeren AM, Scaria A, Schluchter MD, Ferkol TW, Wadsworth S, Davis PB.

Am J Physiol Lung Cell Mol Physiol. 2004 Apr;286(4):L717-26. Epub 2003 Sep 26.


Mouse models of chronic lung infection with Pseudomonas aeruginosa: models for the study of cystic fibrosis.

Stotland PK, Radzioch D, Stevenson MM.

Pediatr Pulmonol. 2000 Nov;30(5):413-24. Review.


A proinflammatory, antiapoptotic phenotype underlies the susceptibility to acute pancreatitis in cystic fibrosis transmembrane regulator (-/-) mice.

Dimagno MJ, Lee SH, Hao Y, Zhou SY, McKenna BJ, Owyang C.

Gastroenterology. 2005 Aug;129(2):665-81.


Iron Homeostasis and Inflammatory Status in Mice Deficient for the Cystic Fibrosis Transmembrane Regulator.

Deschemin JC, Allouche S, Brouillard F, Vaulont S.

PLoS One. 2015 Dec 28;10(12):e0145685. doi: 10.1371/journal.pone.0145685. eCollection 2015.


Spontaneous rescue from cystic fibrosis in a mouse model.

Charizopoulou N, Wilke M, Dorsch M, Bot A, Jorna H, Jansen S, Stanke F, Hedrich HJ, de Jonge HR, Tümmler B.

BMC Genet. 2006 Mar 29;7:18.


Preclinical evidence that sildenafil and vardenafil activate chloride transport in cystic fibrosis.

Lubamba B, Lecourt H, Lebacq J, Lebecque P, De Jonge H, Wallemacq P, Leal T.

Am J Respir Crit Care Med. 2008 Mar 1;177(5):506-15. Epub 2007 Nov 15.


Reduced expression of Tis7/IFRD1 protein in murine and human cystic fibrosis airway epithelial cell models homozygous for the F508del-CFTR mutation.

Blanchard E, Marie S, Riffault L, Bonora M, Tabary O, Clement A, Jacquot J.

Biochem Biophys Res Commun. 2011 Aug 5;411(3):471-6. doi: 10.1016/j.bbrc.2011.06.104. Epub 2011 Jun 24.


An essential role for resident fibroblasts in experimental lung fibrosis is defined by lineage-specific deletion of high-affinity type II transforming growth factor β receptor.

Hoyles RK, Derrett-Smith EC, Khan K, Shiwen X, Howat SL, Wells AU, Abraham DJ, Denton CP.

Am J Respir Crit Care Med. 2011 Jan 15;183(2):249-61. doi: 10.1164/rccm.201002-0279OC. Epub 2010 Aug 13.


Therapeutic effect of a peptide inhibitor of TGF-β on pulmonary fibrosis.

Arribillaga L, Dotor J, Basagoiti M, Riezu-Boj JI, Borrás-Cuesta F, Lasarte JJ, Sarobe P, Cornet ME, Feijoó E.

Cytokine. 2011 Mar;53(3):327-33. doi: 10.1016/j.cyto.2010.11.019. Epub 2010 Dec 23.


Pharmacological modulation of cGMP levels by phosphodiesterase 5 inhibitors as a therapeutic strategy for treatment of respiratory pathology in cystic fibrosis.

Poschet JF, Timmins GS, Taylor-Cousar JL, Ornatowski W, Fazio J, Perkett E, Wilson KR, Yu HD, de Jonge HR, Deretic V.

Am J Physiol Lung Cell Mol Physiol. 2007 Sep;293(3):L712-9. Epub 2007 Jun 22.


Pivotal role of connective tissue growth factor in lung fibrosis: MAPK-dependent transcriptional activation of type I collagen.

Ponticos M, Holmes AM, Shi-wen X, Leoni P, Khan K, Rajkumar VS, Hoyles RK, Bou-Gharios G, Black CM, Denton CP, Abraham DJ, Leask A, Lindahl GE.

Arthritis Rheum. 2009 Jul;60(7):2142-55. doi: 10.1002/art.24620.


New microbicidal functions of tracheal glands: defective anti-infectious response to Pseudomonas aeruginosa in cystic fibrosis.

Bastonero S, Le Priol Y, Armand M, Bernard CS, Reynaud-Gaubert M, Olive D, Parzy D, de Bentzmann S, Capo C, Mege JL.

PLoS One. 2009;4(4):e5357. doi: 10.1371/journal.pone.0005357. Epub 2009 Apr 28.

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