title: Role of IRE1α/XBP-1 in Cystic Fibrosis Airway Inflammation.
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.
authors.affiliation: Marsico Lung Institute/Cystic Fibrosis Research Center, The University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA. carla_ribeiro@med.unc.edu.|Department of Medicine, The University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA. carla_ribeiro@med.unc.edu.|Department of Cell Biology and Physiology, The University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA. carla_ribeiro@med.unc.edu.
authors.forename: Carla M P
authors.initials: CM
authors.lastname: Ribeiro
authors.affiliation: Marsico Lung Institute/Cystic Fibrosis Research Center, The University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA. lubamba@med.unc.edu.
authors.forename: Bob A
authors.initials: BA
authors.lastname: Lubamba
mesh_terms: D003550:Cystic Fibrosis / Q000473:pathology*; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000235:genetics; D006801:Humans; D007249:Inflammation / Q000473:pathology*; D017346:Protein Serine-Threonine Kinases / Q000378:metabolism*; D012137:Respiratory System / Q000473:pathology*; D056811:Unfolded Protein Response
chemical_list: D019005:Cystic Fibrosis Transmembrane Conductance Regulator; D017346:Protein Serine-Threonine Kinases
keywords: CFTR; IRE1α; UPR; XBP-1; airway epithelia; airway inflammation; airway macrophage; cystic fibrosis; innate immunity