Cystic fibrosis: a mucosal immunodeficiency syndrome

TS Cohen, A Prince - Nature medicine, 2012 - nature.com
Cystic fibrosis transmembrane conductance regulator (CFTR) functions as a channel that
regulates the transport of ions and the movement of water across the epithelial barrier …

Human cellular models for the investigation of lung inflammation and mucus production in cystic fibrosis

S Castellani, S Di Gioia, L di Toma… - Analytical Cellular …, 2018 - Wiley Online Library
Chronic inflammation, oxidative stress, mucus plugging, airway remodeling, and respiratory
infections are the hallmarks of the cystic fibrosis (CF) lung disease. The airway epithelium is …

Expression of microRNA-93 and Interleukin-8 during Pseudomonas aeruginosa–Mediated Induction of Proinflammatory Responses

E Fabbri, M Borgatti, G Montagner… - American journal of …, 2014 - atsjournals.org
In this study we analyzed the microRNA profile of cystic fibrosis (CF) bronchial epithelial IB3–
1 cells infected with Pseudomonas aeruginosa by microarray and quantitative RT-PCR …

Pyrogallol, an active compound from the medicinal plant Emblica officinalis, regulates expression of pro-inflammatory genes in bronchial epithelial cells

E Nicolis, I Lampronti, MC Dechecchi, M Borgatti… - International …, 2008 - Elsevier
The most relevant cause of morbidity and mortality in cystic fibrosis (CF) patients is the lung
pathology characterized by chronic infection and inflammation sustained mainly by …

[HTML][HTML] The NFκB signaling in cystic fibrosis lung disease: pathophysiology and therapeutic potential

M Bodas, N Vij - Discovery medicine, 2010 - ncbi.nlm.nih.gov
Lung disease is the major cause of morbidity and mortality of cystic fibrosis (CF), an
autosomal recessive disease caused by mutations in CF transmembrane-conductance …

[HTML][HTML] Mechanisms of the noxious inflammatory cycle in cystic fibrosis

M Rottner, JM Freyssinet, MC Martínez - Respiratory research, 2009 - Springer
Multiple evidences indicate that inflammation is an event occurring prior to infection in
patients with cystic fibrosis. The self-perpetuating inflammatory cycle may play a pathogenic …

[HTML][HTML] Pharmacological therapy for cystic fibrosis: from bench to bedside

F Becq, MA Mall, DN Sheppard, M Conese… - Journal of Cystic …, 2011 - Elsevier
With knowledge of the molecular behaviour of the cystic fibrosis transmembrane
conductance regulator (CFTR), its physiological role and dysfunction in cystic fibrosis (CF) …

Pseudomonas aeruginosa suppresses interferon response to rhinovirus infection in cystic fibrosis but not in normal bronchial epithelial cells

SS Chattoraj, S Ganesan, A Faris… - Infection and …, 2011 - Am Soc Microbiol
Despite increased morbidity associated with secondary respiratory viral infections in cystic
fibrosis (CF) patients with chronic Pseudomonas aeruginosa infection, the underlying …

[HTML][HTML] Pseudomonas aeruginosa PA5oct Jumbo Phage Impacts Planktonic and Biofilm Population and Reduces Its Host Virulence

T Olszak, K Danis-Wlodarczyk, M Arabski, G Gula… - Viruses, 2019 - mdpi.com
The emergence of phage-resistant mutants is a key aspect of lytic phages-bacteria
interaction and the main driver for the co-evolution between both organisms. Here, we …

[HTML][HTML] Expression of wild-type CFTR suppresses NF-κB-driven inflammatory signalling

MJ Hunter, KJ Treharne, AK Winter, DM Cassidy… - PloS one, 2010 - journals.plos.org
Background Mutation of the cystic fibrosis transmembrane-conductance regulator (CFTR)
causes cystic fibrosis (CF) but not all CF aspects can easily be explained by deficient ion …