Atypical Activation of the Unfolded Protein Response in Cystic Fibrosis Airway Cells Contributes to p38 MAPK-Mediated Innate Immune Responses

• Published in: The Journal of immunology (1950) Vol. 189; no. 11; pp. 5467 - 5475
• Main Authors: Blohmke, Christoph J, Mayer, Matthew L, Tang, Anthony C, Hirschfeld, Aaron F, Fjell, Christopher D, Sze, Marc A, Falsafi, Reza, Wang, Shirley, Hsu, Karolynn, Chilvers, Mark A, Hogg, James C, Hancock, Robert E W, Turvey, Stuart E
• Format: Journal Article
• Language: English
• Published: United States 01.12.2012
• Subjects: Cells, Cultured; Cinnamates - pharmacology; Cystic Fibrosis - complications; Cystic Fibrosis - immunology; Cystic Fibrosis - pathology; eIF-2 Kinase - genetics; eIF-2 Kinase - immunology; Endoplasmic Reticulum - drug effects; Endoplasmic Reticulum Stress - drug effects; Epithelial Cells - immunology; Epithelial Cells - pathology; Eukaryotic Initiation Factor-2 - genetics; Eukaryotic Initiation Factor-2 - immunology; Flagellin - immunology; Flagellin - pharmacology; Gene Expression Profiling; Gene Expression Regulation - drug effects; Gene Expression Regulation - immunology; Humans; Immunity, Innate - drug effects; Interleukin-6 - biosynthesis; Interleukin-6 - immunology; Lung - immunology; Lung - pathology; p38 Mitogen-Activated Protein Kinases - genetics; p38 Mitogen-Activated Protein Kinases - immunology; Pneumonia - complications; Pneumonia - immunology; Pneumonia - pathology; Pseudomonas aeruginosa; Pseudomonas aeruginosa - immunology; Signal Transduction - drug effects; Thiourea - analogs & derivatives; Thiourea - pharmacology; Unfolded Protein Response - drug effects; Unfolded Protein Response - immunology

Inflammatory lung disease is the major cause of morbidity and mortality in cystic fibrosis (CF); understanding what produces dysregulated innate immune responses in CF cells will be pivotal in guiding the development of novel anti-inflammatory therapies. To elucidate the molecular mechanisms that me...