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Nat Rev Immunol. 2017 Jun;17(6):363-375. doi: 10.1038/nri.2017.21. Epub 2017 Apr 10.

Mitochondrial DNA in innate immune responses and inflammatory pathology.

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Department of Microbial Pathogenesis and Immunology, Texas A&M University Health Science Center, 470 Reynolds Medical Building, TAMU 1114, College Station, Texas 77843, USA.
Departments of Pathology and Genetics and Yale Center for Research on Aging, Yale School of Medicine, 310 Cedar Street, BML 371, New Haven, Connecticut 06520, USA.


Mitochondrial DNA (mtDNA) - which is well known for its role in oxidative phosphorylation and maternally inherited mitochondrial diseases - is increasingly recognized as an agonist of the innate immune system that influences antimicrobial responses and inflammatory pathology. On entering the cytoplasm, extracellular space or circulation, mtDNA can engage multiple pattern-recognition receptors in cell-type- and context-dependent manners to trigger pro-inflammatory and type I interferon responses. Here, we review the expanding research field of mtDNA in innate immune responses to highlight new mechanistic insights and discuss the physiological and pathological relevance of this exciting area of mitochondrial biology.

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