Role of IRE1α/XBP-1 in Cystic Fibrosis Airway Inflammation

Autor: Carla M. P. Ribeiro, Bob A. Lubamba
Jazyk: angličtina
Rok vydání: 2017
Předmět:
Zdroj: International Journal of Molecular Sciences, Vol 18, Iss 1, p 118 (2017)
Druh dokumentu: article
ISSN: 1422-0067
DOI: 10.3390/ijms18010118
Popis: 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.
Databáze: Directory of Open Access Journals