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John Paul Clancy

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Kong M, Whitley R, Peng N, Oster R, Schoeb T, Sullender W, et al.
Viruses . 2015 Aug; 7(8):4230-53. PMID: 26264019
Respiratory Syncytial Virus (RSV) is an important human pathogen associated with substantial morbidity and mortality. The present study tested the hypothesis that RSV infection would increase matrix metalloproteinase (MMP)-9 expression,...
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Accurso F, Van Goor F, Zha J, Stone A, Dong Q, Ordonez C, et al.
J Cyst Fibros . 2014 Mar; 13(2):139-47. PMID: 24660233
Background: We examined data from a Phase 2 trial {NCT00457821} of ivacaftor, a CFTR potentiator, in cystic fibrosis (CF) patients with aG551D mutation to evaluate standardized approaches to sweat chloride...
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Kong M, Maeng P, Hong J, Szczesniak R, Sorscher E, Sullender W, et al.
Viruses . 2013 Sep; 5(9):2260-71. PMID: 24056672
Background: Respiratory Syncytial Virus (RSV) infection is a common contributor to pulmonary symptoms in children with cystic fibrosis (CF). Here we examined RSV infection in immortalized bronchial epithelial cells (CFBE41o-)...
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Rowe S, Clancy J, Wilschanski M
Methods Mol Biol . 2011 May; 741:69-86. PMID: 21594779
The Nasal potential difference measurement is used to measure the voltage across the nasal epithelium, which results from transepithelial ion transport and reflects in part CFTR function. The electrophysiologic abnormality...
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Solomon G, Konstan M, Wilschanski M, Billings J, Sermet-Gaudelus I, Accurso F, et al.
Chest . 2010 May; 138(4):919-28. PMID: 20472865
Background: The transepithelial nasal potential difference (NPD) is used to assess cystic fibrosis transmembrane conductance regulator (CFTR) activity. Unreliability, excessive artifacts, and lack of standardization of current testing systems can...
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Jackson P, Xu X, Wilson L, Weathington N, Clancy J, Blalock J, et al.
Mol Med . 2010 Jan; 16(5-6):159-66. PMID: 20111696
Cystic fibrosis (CF) is a lethal genetic disorder characterized by airway remodeling and inflammation, leading to premature death. Recent evidence suggests the importance of protease activity in CF pathogenesis. One...
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Chen L, Bosworth C, Pico T, Collawn J, Varga K, Gao Z, et al.
Am J Respir Cell Mol Biol . 2008 Mar; 39(2):150-62. PMID: 18314534
We investigated the cellular mechanisms by which nitric oxide (NO) increases chloride (Cl-) secretion across lung epithelial cells in vitro and in vivo. Addition of (Z)-1-[2-(2-aminoethyl)-N-(2-ammonioethyl) amino] diazen-1-ium-1, 2-diolate (DETANONOate...
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Rennolds J, Tower C, Musgrove L, Fan L, Maloney K, Clancy J, et al.
J Biol Chem . 2007 Oct; 283(2):833-9. PMID: 17932045
Cystic fibrosis (CF) is caused by defects in the CF transmembrane conductance regulator (CFTR) that functions as a chloride channel in epithelial cells. The most common cause of CF is...
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Wang W, Li G, Clancy J, Kirk K
J Biol Chem . 2005 Apr; 280(25):23622-30. PMID: 15857825
Cystic fibrosis (CF) is caused by mutations that disrupt the surface localization and/or gating of the CF transmembrane conductance regulator (CFTR) chloride channel. The most common CF mutant is deltaF508-CFTR,...