- Interactions between infection and inflammation in the cystic fibrosis airway

2015 ◽  
pp. 114-134
Keyword(s):  
Cystic Fibrosis ◽  
Cystic Fibrosis Airway ◽  
Infection And Inflammation

scholarly journals Physiological Impact of Abnormal Lipoxin A4Production on Cystic Fibrosis Airway Epithelium and Therapeutic Potential

2015 ◽  
Vol 2015 ◽  
pp. 1-10 ◽  
Author(s):  
Gerard Higgins ◽  
Fiona Ringholz ◽  
Paul Buchanan ◽  
Paul McNally ◽  
Valérie Urbach
Keyword(s):  
Cystic Fibrosis ◽  
Therapeutic Potential ◽  
Chloride Transport ◽  
Early Death ◽  
Airway Epithelial ◽  
Resolution Of Inflammation ◽  
Cystic Fibrosis Airway ◽  
Infection And Inflammation ◽  
Subsequent Activation ◽  
Physiological Impact

Lipoxin A4has been described as a major signal for the resolution of inflammation and is abnormally produced in the lungs of patients with cystic fibrosis (CF). In CF, the loss of chloride transport caused by the mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) Cl−channel gene results in dehydration, mucus plugging, and reduction of the airway surface liquid layer (ASL) height which favour chronic lung infection and neutrophil based inflammation leading to progressive lung destruction and early death of people with CF. This review highlights the unique ability of LXA4to restore airway surface hydration, to stimulate airway epithelial repair, and to antagonise the proinflammatory program of the CF airway, circumventing some of the most difficult aspects of CF pathophysiology. The report points out novel aspects of the cellular mechanism involved in the physiological response to LXA4, including release of ATP from airway epithelial cell via pannexin channel and subsequent activation of and P2Y11 purinoreceptor. Therefore, inadequate endogenous LXA4biosynthesis reported in CF exacerbates the ion transport abnormality and defective mucociliary clearance, in addition to impairing the resolution of inflammation, thus amplifying the vicious circle of airway dehydration, chronic infection, and inflammation.

scholarly journals Targeted Drug Delivery Technologies Potentiate the Overall Therapeutic Efficacy of an Indole Derivative in a Mouse Cystic Fibrosis Setting

Cells ◽  
2021 ◽  
Vol 10 (7) ◽  
pp. 1601
Author(s):  
Matteo Puccetti ◽  
Marilena Pariano ◽  
Giorgia Renga ◽  
Ilaria Santarelli ◽  
Fiorella D’Onofrio ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Targeted Drug Delivery ◽  
Fungal Infections ◽  
Murine Models ◽  
Multisystem Disease ◽  
Aryl Hydrocarbon ◽  
Target Organs ◽  
Infection And Inflammation ◽  
Anti Inflammatory ◽  
Inflammatory Toxicity

Inflammation plays a major role in the pathophysiology of cystic fibrosis (CF), a multisystem disease. Anti-inflammatory therapies are, therefore, of interest in CF, provided that the inhibition of inflammation does not compromise the ability to fight pathogens. Here, we assess whether indole-3-aldehyde (3-IAld), a ligand of the aryl hydrocarbon receptor (AhR), may encompass such an activity. We resorted to biopharmaceutical technologies in order to deliver 3-IAld directly into the lung, via dry powder inhalation, or into the gut, via enteric microparticles, in murine models of CF infection and inflammation. We found the site-specific delivery of 3-IAld to be an efficient strategy to restore immune and microbial homeostasis in CF organs, and mitigate lung and gut inflammatory pathology in response to fungal infections, in the relative absence of local and systemic inflammatory toxicity. Thus, enhanced delivery to target organs of AhR agonists, such as 3-IAld, may pave the way for the development of safe and effective anti-inflammatory agents in CF.

scholarly journals Small-molecule ion channels increase host defences in cystic fibrosis airway epithelia

Nature ◽  
2019 ◽  
Vol 567 (7748) ◽  
pp. 405-408 ◽  
Author(s):  
Katrina A. Muraglia ◽  
Rajeev S. Chorghade ◽  
Bo Ram Kim ◽  
Xiao Xiao Tang ◽  
Viral S. Shah ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Ion Channels ◽  
Small Molecule ◽  
Airway Epithelia ◽  
Cystic Fibrosis Airway

Inflammation and Infection in Naive Human Cystic Fibrosis Airway Grafts

2000 ◽  
Vol 23 (2) ◽  
pp. 121-127 ◽  
Author(s):  
Rabindra Tirouvanziam ◽  
Sophie de Bentzmann ◽  
Cédric Hubeau ◽  
Jocelyne Hinnrasky ◽  
Jacky Jacquot ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Cystic Fibrosis Airway

Inefficient gene transfer by adenovirus vector to cystic fibrosis airway epithelia of mice and humans

Nature ◽  
1994 ◽  
Vol 371 (6500) ◽  
pp. 802-806 ◽  
Author(s):  
Barbara R. Grubb ◽  
Raymond J. Pickles ◽  
Hong Ye ◽  
James R. Yankaskas ◽  
Ralph N. Vick ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Gene Transfer ◽  
Adenovirus Vector ◽  
Airway Epithelia ◽  
Cystic Fibrosis Airway

scholarly journals Pseudomonas aeruginosa biofilm formation in the cystic fibrosis airway

2008 ◽  
Vol 21 (4) ◽  
pp. 595-599 ◽  
Author(s):  
Sophie Moreau-Marquis ◽  
Bruce A. Stanton ◽  
George A. O’Toole
Keyword(s):  
Cystic Fibrosis ◽  
Pseudomonas Aeruginosa ◽  
Biofilm Formation ◽  
Cystic Fibrosis Airway
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