Background: Periodic fever syndromes are caused by deregulation of Interleukin-1β release. Results: Defective autophagy leads to accumulation of damaged mitochondria in monocytes. Conclusion: Mitochondrial components in the cytosol cause priming of monocytes for Interleukin-1β release. Significance: The molecular mechanism behind deregulated cytokine secretion provides new clues for intervention. © 2014 by The American Society for Biochemistry and Molecular Biology, Inc.
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