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 …

[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 …

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 …

Cystic fibrosis macrophage function and clinical outcomes after elexacaftor/tezacaftor/ivacaftor

S Zhang, CL Shrestha, F Robledo-Avila… - European …, 2023 - Eur Respiratory Soc
Background Abnormal macrophage function caused by dysfunctional cystic fibrosis
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

S Zhang, CL Shrestha, BT Kopp - Scientific reports, 2018 - nature.com
Despite the addition of cystic fibrosis transmembrane conductance regulator (CFTR)
modulators to the cystic fibrosis (CF) treatment regimen, patients with CF continue to suffer …

Cystic fibrosis: insight into CFTR pathophysiology and pharmacotherapy

B Lubamba, B Dhooghe, S Noel, T Leal - Clinical biochemistry, 2012 - Elsevier
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 …

Pharmacological modulation of the AKT/microRNA-199a-5p/CAV1 pathway ameliorates cystic fibrosis lung hyper-inflammation

P Zhang, J Cheng, S Zou, AD D'Souza, JL Koff… - Nature …, 2015 - nature.com
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 …

Transcriptional and functional diversity of human macrophage repolarization

SA Gharib, RS McMahan, WE Eddy, ME Long… - Journal of Allergy and …, 2019 - Elsevier
Background Macrophage plasticity allows cells to adopt different phenotypes, a property
with important implications in disorders such as cystic fibrosis (CF) and asthma. Objective …

Strategies for the etiological therapy of cystic fibrosis

L Maiuri, V Raia, G Kroemer - Cell Death & Differentiation, 2017 - nature.com
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) …

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 …