Inflammation and its genesis in cystic fibrosis
DP Nichols, JF Chmiel - Pediatric pulmonology, 2015 - Wiley Online Library
The host inflammatory response in cystic fibrosis (CF) lung disease has long been
recognized as a central pathological feature and an important therapeutic target. Indeed …
recognized as a central pathological feature and an important therapeutic target. Indeed …
[HTML][HTML] Innate immunity in cystic fibrosis lung disease
D Hartl, A Gaggar, E Bruscia, A Hector, V Marcos… - Journal of Cystic …, 2012 - Elsevier
Chronic lung disease determines the morbidity and mortality of cystic fibrosis (CF) patients.
The pulmonary immune response in CF is characterized by an early and non-resolving …
The pulmonary immune response in CF is characterized by an early and non-resolving …
Cystic fibrosis lung immunity: the role of the macrophage
EM Bruscia, TL Bonfield - Journal of innate immunity, 2016 - karger.com
Cystic fibrosis (CF) pathophysiology is hallmarked by excessive inflammation and the
inability to efficiently resolve lung infections, contributing to major morbidity and eventually …
inability to efficiently resolve lung infections, contributing to major morbidity and eventually …
Cystic fibrosis macrophage function and clinical outcomes after elexacaftor/tezacaftor/ivacaftor
Background Abnormal macrophage function caused by dysfunctional cystic fibrosis
transmembrane conductance regulator (CFTR) is a critical contributor to chronic airway …
transmembrane conductance regulator (CFTR) is a critical contributor to chronic airway …
Cystic fibrosis transmembrane conductance regulator (CFTR) modulators have differential effects on cystic fibrosis macrophage function
Despite the addition of cystic fibrosis transmembrane conductance regulator (CFTR)
modulators to the cystic fibrosis (CF) treatment regimen, patients with CF continue to suffer …
modulators to the cystic fibrosis (CF) treatment regimen, patients with CF continue to suffer …
Cystic fibrosis: insight into CFTR pathophysiology and pharmacotherapy
Cystic fibrosis is the most common life-threatening recessively inherited disease in
Caucasians. Due to early provision of care in specialized reference centers and more …
Caucasians. Due to early provision of care in specialized reference centers and more …
Pharmacological modulation of the AKT/microRNA-199a-5p/CAV1 pathway ameliorates cystic fibrosis lung hyper-inflammation
In cystic fibrosis (CF) patients, hyper-inflammation is a key factor in lung destruction and
disease morbidity. We have previously demonstrated that macrophages drive the lung hyper …
disease morbidity. We have previously demonstrated that macrophages drive the lung hyper …
Transcriptional and functional diversity of human macrophage repolarization
Background Macrophage plasticity allows cells to adopt different phenotypes, a property
with important implications in disorders such as cystic fibrosis (CF) and asthma. Objective …
with important implications in disorders such as cystic fibrosis (CF) and asthma. Objective …
Strategies for the etiological therapy of cystic fibrosis
Etiological therapies aim at repairing the underlying cause of cystic fibrosis (CF), which is
the functional defect of the cystic fibrosis transmembrane conductance regulator (CFTR) …
the functional defect of the cystic fibrosis transmembrane conductance regulator (CFTR) …
Mutations of cystic fibrosis transmembrane conductance regulator gene cause a monocyte-selective adhesion deficiency
C Sorio, A Montresor, M Bolomini-Vittori… - American journal of …, 2016 - atsjournals.org
Rationale: Cystic fibrosis (CF) is a common genetic disease caused by mutations of the
cystic fibrosis transmembrane conductance regulator (CFTR) gene. Persistent lung …
cystic fibrosis transmembrane conductance regulator (CFTR) gene. Persistent lung …