Cystic Fibrosis
|
0.100 |
Biomarker
|
disease |
BEFREE |
We aimed to compare different techniques to detect active NE in sputum samples of 50 Bronchiectasis (BE) and 50 Cystic Fibrosis (CF) patients.
|
31626976 |
2019 |
Cystic Fibrosis
|
0.100 |
Biomarker
|
disease |
BEFREE |
Neutrophils are major drivers of cystic fibrosis (CF) lung disease, in part due to the release of human neutrophil elastase- and myeloperoxidase-rich primary granules, leading to tissue damage.
|
29937317 |
2019 |
Cystic Fibrosis
|
0.100 |
Biomarker
|
disease |
BEFREE |
The lead NSGM so identified neutralized neutrophil elastase present in the sputum of CF patients in the presence of deoxyribonuclease and high-salt conditions.
|
31074986 |
2019 |
Cystic Fibrosis
|
0.100 |
Biomarker
|
disease |
BEFREE |
Cystic fibrosis (CF) lung disease is characterized by aggressive neutrophil-dominated inflammation mediated in large part by neutrophil elastase (NE), an omnivorous protease released by activated or disintegrating neutrophils and a key therapeutic target.
|
29960875 |
2019 |
Cystic Fibrosis
|
0.100 |
Biomarker
|
disease |
BEFREE |
Inhalation of POL6014 can safely lead to high concentrations within the lung and simultaneously low plasma concentrations, allowing for a clear inhibition of NE in the sputum of subjects with CF after single dosing.
|
31501052 |
2019 |
Cystic Fibrosis
|
0.100 |
Biomarker
|
disease |
BEFREE |
Stability of SPLUNC1, S18 (the ENaC regulatory domain of SPLUNC1), and SPX-101 was determined in sputum from CF donors and towards neutrophil elastase.
|
29936069 |
2019 |
Cystic Fibrosis
|
0.100 |
Biomarker
|
disease |
BEFREE |
Safety and efficacy of the human neutrophil elastase inhibitor BAY 85-8501 for the treatment of non-cystic fibrosis bronchiectasis: A randomized controlled trial.
|
30917927 |
2019 |
Cystic Fibrosis
|
0.100 |
Biomarker
|
disease |
BEFREE |
Neutrophil elastase correlates with increased sphingolipid content in cystic fibrosis sputum.
|
29624923 |
2018 |
Cystic Fibrosis
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
Lung disease in patients with CF is associated with neutrophil-dominated inflammation and elevated levels of the serine protease, neutrophil elastase (NE).
|
29401615 |
2018 |
Cystic Fibrosis
|
0.100 |
Biomarker
|
disease |
BEFREE |
Our hypothesis is that variants of heparin, which potently inhibit NE but are not anticoagulant, would help restore the protease-antiprotease balance in CF.
|
29903912 |
2018 |
Cystic Fibrosis
|
0.100 |
Biomarker
|
disease |
BEFREE |
Our results suggest that surface-bound NE activity may play an important role in the pathogenesis and serve as novel biomarker in CF lung disease.
|
29545279 |
2018 |
Cystic Fibrosis
|
0.100 |
Biomarker
|
disease |
BEFREE |
<i>Pseudomonas</i> on BAL was associated with positive neutrophil elastase (OR 4.17, 95% CI 2.04-8.53; p<0.001), increased interleukin-8 (p<0.001), increased all baseline PRAGMA computed tomography scores (p<0.001), progression of PRAGMA computed tomography scores (p<0.05) and increased risk of respiratory exacerbations (incidence rate ratio 2.11, 95% CI 1.15-3.87; p=0.017).In children with CF OPSs only marginally change the probability of detecting lower airway <i>Pseudomonas</i> and are not associated with lung disease indices nor exacerbations risk.
|
29678944 |
2018 |
Cystic Fibrosis
|
0.100 |
Biomarker
|
disease |
BEFREE |
Plasma from 17 patients with CF (stable and pre and post pulmonary exacerbation) and eight healthy volunteers was analyzed for sTREM-1 and proteases (matrix metalloproteinase-8 (MMP-8), MMP-9, and human neutrophil elastase HNE).
|
28068000 |
2017 |
Cystic Fibrosis
|
0.100 |
Biomarker
|
disease |
BEFREE |
C5a levels also correlated positively with concentrations of other sputum markers associated with worse CF lung disease including neutrophil elastase (P < 0.001), myeloperoxidase activity (P = 0.006) and DNA concentration (P < 0.001).
|
28278205 |
2017 |
Cystic Fibrosis
|
0.100 |
Biomarker
|
disease |
BEFREE |
In contrast, MUC5B D-domains were modified by neutrophil elastase, a protease commonly found in CF sputum, demonstrating that proteolytic degradation of MUC5B is an extracellular event in CF sputum.
|
26993521 |
2016 |
Cystic Fibrosis
|
0.100 |
Biomarker
|
disease |
BEFREE |
Both variants showed enhanced resistance to degradation in the presence of excess NE as well as CF patient sputum compared with SLPI-wild type (SLPI-WT).
|
26376365 |
2016 |
Cystic Fibrosis
|
0.100 |
Biomarker
|
disease |
BEFREE |
Our results suggest that NE plays an important role in the in vivo pathogenesis and may serve as a therapeutic target for inflammation, mucus hypersecretion, and structural lung damage and indicate that additional rehydration strategies may be required for effective treatment of airway mucus obstruction in CF.
|
24678594 |
2014 |
Cystic Fibrosis
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
In contrast to neutrophil elastase, CTSS activity was detectable in 100% of CF BAL fluid samples from patients without Pseudomonas aeruginosa infection.
|
24940638 |
2014 |
Cystic Fibrosis
|
0.100 |
Biomarker
|
disease |
BEFREE |
Unlike control, CF human bronchial epithelial cultures (HBECs) where airway surface liquid (ASL) height was abnormally low (4.2 ± 0.6 μm), addition of S18 prevented ENaC-led ASL hyperabsorption and maintained CF ASL height at 7.9 ± 0.6 μm, even in the presence of neutrophil elastase, which is comparable to heights seen in normal HBECs.
|
24124190 |
2013 |
Cystic Fibrosis
|
0.100 |
Biomarker
|
disease |
BEFREE |
We hypothesized that the high load of NE in the CF airway triggers epithelial senescence by upregulating expression of p16, which inhibits cyclin-dependent kinase 4 (CDK4).
|
23316069 |
2013 |
Cystic Fibrosis
|
0.100 |
Biomarker
|
disease |
BEFREE |
We have shown that the active neutrophil elastase, protease 3, and cathepsin G in CF sputum resist inhibition in part by exogenous protease inhibitors.
|
22343221 |
2012 |
Cystic Fibrosis
|
0.100 |
Biomarker
|
disease |
BEFREE |
Here, we show that the Pseudomonas proteases (Pseudomonas elastase and alkaline protease) and the neutrophil proteases (neutrophil elastase (NE) and proteinase-3) are capable of almost complete degradation of hemoglobin in vitro but that NE is the predominant protease that cleaves hemoglobin in vivo in CF bronchoalveolar lavage fluid.
|
21193404 |
2011 |
Cystic Fibrosis
|
0.100 |
Biomarker
|
disease |
BEFREE |
The antimicrobial and hemolytic activities of a host defense peptide can be controlled by its modification as a propeptide of reduced net charge, which can then be processed by neutrophil elastase, a serine protease involved in chronic airway inflammation and infections associated with cystic fibrosis.
|
21343449 |
2011 |
Cystic Fibrosis
|
0.100 |
Biomarker
|
disease |
BEFREE |
Excess neutrophil elastase is an important determinant of pulmonary disease in CF. alpha1-antitrypsin (AAT), also known as alpha1-proteinase inhibitor (alpha1PI) is a major modulator of elastase activity.
|
11313771 |
2001 |
Cystic Fibrosis
|
0.100 |
AlteredExpression
|
disease |
BEFREE |
There was also a clear association between CF ELF active NE and IL-8 levels (r = 0.94).
|
1357002 |
1992 |