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J Interferon Cytokine Res. 2017 May;37(5):207-213. doi: 10.1089/jir.2016.0095.

RIG-I-Like Receptors and Type I Interferonopathies.

Author information

1
Laboratory of Molecular Genetics, Institute for Virus Research, Kyoto University , Kyoto, Japan .

Abstract

Type I interferon (IFN) production by the proper activation of nucleic acid sensors is essential for hosts to eliminate invading viruses. Among these sensors, RIG-I-like receptors (RLRs) are well-known viral RNA sensors in the cytoplasm that recognize the nonself signatures of viral RNAs to trigger IFN responses. Recent accumulating evidence has clarified that some specific and atypical self-RNAs also cause activation of RLRs independently of virus infection. Importantly, when RLR-activation by these RNAs or a conformational change via missense mutations is sustained, the resulting continuous production of type I IFN will lead to autoimmune disorders. We, herein, focus on autoimmune diseases caused by chronic activation of RLRs and discuss possible mechanisms of their onset.

KEYWORDS:

RIG-I-like receptors (RLRs); autoimmune diseases; interferon (IFN)

PMID:
28475461
PMCID:
PMC5439449
DOI:
10.1089/jir.2016.0095
[Indexed for MEDLINE]
Free PMC Article

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