Ribeiro Carla M P, Lubamba Bob A
Marsico Lung Institute/Cystic Fibrosis Research Center, The University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.
Department of Medicine, The University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA.
Int J Mol Sci. 2017 Jan 9;18(1):118. doi: 10.3390/ijms18010118.
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.
囊性纤维化（CF）肺部疾病的特征是慢性气道感染和炎症。感染性和炎症性的CF气道环境会影响气道上皮细胞和气道巨噬细胞的固有防御功能。CF气道环境会诱导这些细胞发生适应性变化，其特征是基础炎症增加以及对炎症介质产生强烈的炎症反应。最近的研究表明，这些反应依赖于未折叠蛋白反应（UPR）的激活。本综述讨论了气道上皮细胞和气道巨噬细胞对CF气道炎症反应的作用，并特别强调了由IRE1/XBP-1介导的UPR途径在这些过程中的功能重要性。这些发现表明，靶向IRE1/XBP-1 UPR途径可能是治疗CF气道疾病的一种策略。
