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The role of Lipoxin A4 in Cystic Fibrosis Lung Disease

In Cystic Fibrosis (CF), mutations of the CFTR gene result in defective Cl(−) secretion and Na(+) hyperabsorption by epithelia which leads to airway lumen dehydration and mucus plugging and favours chronic bacterial colonization, persistent inflammation and progressive lung destruction. Beyond this...

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Detalles Bibliográficos
Autores principales: Urbach, Valérie, Higgins, Gerard, Buchanan, Paul, Ringholz, Fiona
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Research Network of Computational and Structural Biotechnology (RNCSB) Organization 2013
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3962119/
https://www.ncbi.nlm.nih.gov/pubmed/24688726
http://dx.doi.org/10.5936/csbj.201303018
Descripción
Sumario:In Cystic Fibrosis (CF), mutations of the CFTR gene result in defective Cl(−) secretion and Na(+) hyperabsorption by epithelia which leads to airway lumen dehydration and mucus plugging and favours chronic bacterial colonization, persistent inflammation and progressive lung destruction. Beyond this general description, the pathogenesis of CF lung disease remains obscure due to an incomplete understanding of normal innate airway defense. This mini-review aims to highlight the role of the pro-resolution lipid mediator, Lipoxin A(4), which is inadequately produced in CF, on several aspects of innate immunity that are altered in CF airway disease.