The role of Lipoxin A4 in cystic fibrosis lung disease

Abstract

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 A4, which is inadequately produced in CF, on several aspects of innate immunity that are altered in CF airway disease. © 2013 Urbach et al.