| 1 |
35413967 | Systemic bis-phosphinic acid derivative restores chloride transport in Cystic Fibrosis mice. | 2022 Apr 12 |
1 |
| 2 |
34034984 | VIP reduction in the pancreas of F508del homozygous CF mice and early signs of Cystic Fibrosis Related Diabetes (CFRD). | 2021 Sep |
1 |
| 3 |
30874543 | Autophagy suppresses the pathogenic immune response to dietary antigens in cystic fibrosis. | 2019 Mar 15 |
1 |
| 4 |
30969810 | Differential thermostability and response to cystic fibrosis transmembrane conductance regulator potentiators of human and mouse F508del-CFTR. | 2019 Jul 1 |
9 |
| 5 |
31189070 | Augmentation of Cystic Fibrosis Transmembrane Conductance Regulator Function in Human Bronchial Epithelial Cells via SLC6A14-Dependent Amino Acid Uptake. Implications for Treatment of Cystic Fibrosis. | 2019 Dec |
1 |
| 6 |
31251792 | Evaluation of eluforsen, a novel RNA oligonucleotide for restoration of CFTR function in in vitro and murine models of p.Phe508del cystic fibrosis. | 2019 |
1 |
| 7 |
31427400 | ORKAMBI-Mediated Rescue of Mucociliary Clearance in Cystic Fibrosis Primary Respiratory Cultures Is Enhanced by Arginine Uptake, Arginase Inhibition, and Promotion of Nitric Oxide Signaling to the Cystic Fibrosis Transmembrane Conductance Regulator Channel. | 2019 Oct |
1 |
| 8 |
28196856 | Vardenafil reduces macrophage pro-inflammatory overresponses in cystic fibrosis through PDE5- and CFTR-dependent mechanisms. | 2017 Jun 1 |
1 |
| 9 |
26769674 | Genetic deletion of keratin 8 corrects the altered bone formation and osteopenia in a mouse model of cystic fibrosis. | 2016 Apr 1 |
7 |
| 10 |
26976279 | Manipulating proteostasis to repair the F508del-CFTR defect in cystic fibrosis. | 2016 Dec |
5 |
| 11 |
27663991 | Correction of lung inflammation in a F508del CFTR murine cystic fibrosis model by the sphingosine-1-phosphate lyase inhibitor LX2931. | 2016 Nov 1 |
1 |
| 12 |
24974227 | Ibuprofen rescues mutant cystic fibrosis transmembrane conductance regulator trafficking. | 2015 Jan |
2 |
| 13 |
26092868 | Resveratrol increases F508del-CFTR dependent salivary secretion in cystic fibrosis mice. | 2015 Jun 19 |
1 |
| 14 |
24529904 | Enhanced F508del-CFTR channel activity ameliorates bone pathology in murine cystic fibrosis. | 2014 Apr |
3 |
| 15 |
24595473 | Pseudomonas aeruginosa reduces the expression of CFTR via post-translational modification of NHERF1. | 2014 Dec |
1 |
| 16 |
24671173 | Lipoxin A4 and platelet activating factor are involved in E. coli or LPS-induced lung inflammation in CFTR-deficient mice. | 2014 |
1 |
| 17 |
24801204 | Reduced blood pressure of CFTR-F508del carriers correlates with diminished arterial reactivity rather than circulating blood volume in mice. | 2014 |
4 |
| 18 |
24898584 | Cystic fibrosis transmembrane conductance regulator dysfunction in VIP knockout mice. | 2014 Jul 15 |
1 |
| 19 |
25350163 | Restoration of CFTR function in patients with cystic fibrosis carrying the F508del-CFTR mutation. | 2014 |
1 |
| 20 |
25441013 | Discovery of N-(2,4-di-tert-butyl-5-hydroxyphenyl)-4-oxo-1,4-dihydroquinoline-3-carboxamide (VX-770, ivacaftor), a potent and orally bioavailable CFTR potentiator. | 2014 Dec 11 |
2 |
| 21 |
25500839 | Role of IL-1β in experimental cystic fibrosis upon P. aeruginosa infection. | 2014 |
2 |
| 22 |
23290341 | Ventilatory pattern and energy expenditure are altered in cystic fibrosis mice. | 2013 Jul |
1 |
| 23 |
23346057 | Targeting the Intracellular Environment in Cystic Fibrosis: Restoring Autophagy as a Novel Strategy to Circumvent the CFTR Defect. | 2013 |
2 |
| 24 |
23505426 | Characterization of nasal potential difference in cftr knockout and F508del-CFTR mice. | 2013 |
4 |
| 25 |
23768658 | Modification of the salivary secretion assay in F508del mice--the murine equivalent of the human sweat test. | 2013 Dec |
1 |
| 26 |
24204804 | Correction of chloride transport and mislocalization of CFTR protein by vardenafil in the gastrointestinal tract of cystic fibrosis mice. | 2013 |
1 |
| 27 |
24367540 | Important role of platelets in modulating endotoxin-induced lung inflammation in CFTR-deficient mice. | 2013 |
2 |
| 28 |
22679004 | Altered de novo lipogenesis contributes to low adipose stores in cystic fibrosis mice. | 2012 Aug 15 |
1 |
| 29 |
22802588 | Rescue of epithelial HCO3- secretion in murine intestine by apical membrane expression of the cystic fibrosis transmembrane conductance regulator mutant F508del. | 2012 Nov 1 |
5 |
| 30 |
23024633 | PDE5 Inhibitors as Potential Tools in the Treatment of Cystic Fibrosis. | 2012 |
5 |
| 31 |
23284872 | Rescue of murine F508del CFTR activity in native intestine by low temperature and proteasome inhibitors. | 2012 |
3 |
| 32 |
21301926 | Role of CFTR expressed by neutrophils in modulating acute lung inflammation and injury in mice. | 2011 Jul |
1 |
| 33 |
21338920 | Identification of a NBD1-binding pharmacological chaperone that corrects the trafficking defect of F508del-CFTR. | 2011 Feb 25 |
8 |
| 34 |
21723850 | Reduced expression of Tis7/IFRD1 protein in murine and human cystic fibrosis airway epithelial cell models homozygous for the F508del-CFTR mutation. | 2011 Aug 5 |
1 |
| 35 |
19625650 | SUMOylation of tissue transglutaminase as link between oxidative stress and inflammation. | 2009 Aug 15 |
1 |
| 36 |
19878303 | Deletion of Phe508 in the first nucleotide-binding domain of the cystic fibrosis transmembrane conductance regulator increases its affinity for the heat shock cognate 70 chaperone. | 2009 Dec |
4 |
| 37 |
18006891 | Preclinical evidence that sildenafil and vardenafil activate chloride transport in cystic fibrosis. | 2008 Mar 1 |
1 |
| 38 |
18309088 | Parallel improvement of sodium and chloride transport defects by miglustat (n-butyldeoxynojyrimicin) in cystic fibrosis epithelial cells. | 2008 Jun |
4 |
| 39 |
18450781 | Congenital tracheal malformation in cystic fibrosis transmembrane conductance regulator-deficient mice. | 2008 Jul 1 |
3 |
| 40 |
18955860 | Expression of intestinal and lung alkaline sphingomyelinase and neutral ceramidase in cystic fibrosis f508del transgenic mice. | 2008 Nov |
1 |