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Int. J. Mol. Sci. 2017, 18(1), 118; doi:10.3390/ijms18010118

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

1
Marsico Lung Institute/Cystic Fibrosis Research Center, The University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA
2
Department of Medicine, The University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA
3
Department of Cell Biology and Physiology, The University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA
*
Authors to whom correspondence should be addressed.
Academic Editor: Masato Matsuoka
Received: 8 December 2016 / Revised: 3 January 2017 / Accepted: 4 January 2017 / Published: 9 January 2017
(This article belongs to the Special Issue Modulators of Endoplasmic Reticulum Stress 2016)
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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. View Full-Text
Keywords: cystic fibrosis; airway inflammation; airway epithelia; airway macrophage; innate immunity; UPR; IRE1α; XBP-1; CFTR cystic fibrosis; airway inflammation; airway epithelia; airway macrophage; innate immunity; UPR; IRE1α; XBP-1; CFTR
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This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. (CC BY 4.0).

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Ribeiro, C.M.P.; Lubamba, B.A. Role of IRE1α/XBP-1 in Cystic Fibrosis Airway Inflammation. Int. J. Mol. Sci. 2017, 18, 118.

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