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Abstract
Cystic fibrosis (CF) pulmonary disease is characterized by chronic airway infection and inflammation. The infectious and inflamed CF airway environment impacts on the innate defense of airway epithelia and airway macrophages. The CF airway milieu induces an adaptation in these cells characterized by increased basal inflammation and a robust inflammatory response to inflammatory mediators. Recent studies have indicated that these responses depend on activation of the unfolded protein response (UPR). This review discusses the contribution of airway epithelia and airway macrophages to CF airway inflammatory responses and specifically highlights the functional importance of the UPR pathway mediated by IRE1/XBP-1 in these processes. These findings suggest that targeting the IRE1/XBP-1 UPR pathway may be a therapeutic strategy for CF airway disease.
Highlights
Cystic fibrosis (CF) airway disease is characterized by a chronic and robust inflammatory state often termed “hyper-inflammatory”
Evidence indicates that cystic fibrosis (CF) patients have inherited and acquired factors that contribute to abnormal immune regulation, resulting in robust airway inflammation
While previous studies have suggested that cystic fibrosis transmembrane conductance regulator (CFTR) dysfunction confers a pro-inflammatory airway phenotype, other findings, including studies from primary CF airway epithelial cells and macrophages, provide compelling evidence that the exaggerated inflammatory status of CF airways results, instead, from an acquired response to the CF airway milieu
Summary
Cystic fibrosis (CF) airway disease is characterized by a chronic and robust inflammatory state often termed “hyper-inflammatory”. Expression of DN-XBP-1 decreased basal IL-8 secretion and blunted SMM-promoted IL-8 secretion [12] These studies have indicated that the increased XBP-1s levels resulting from SMM exposure are responsible for the ER Ca2+ store expansion, thereby providing a mechanism for the robust airway epithelial IL-8 secretory phenotype induced by the infectious/inflammatory milieu of CF airways [12]. It remains to be established whether the IL-8 promoter contains a binding site for XBP-1s
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