Kato Hiroki, Oh Seong-Wook, Fujita Takashi
Laboratory of Molecular Genetics, Institute for Virus Research, Kyoto University , Kyoto, Japan .
J Interferon Cytokine Res. 2017 May;37(5):207-213. doi: 10.1089/jir.2016.0095.
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.
通过核酸传感器的适当激活产生I型干扰素（IFN）对于宿主消除入侵病毒至关重要。在这些传感器中，RIG-I样受体（RLRs）是细胞质中著名的病毒RNA传感器，可识别病毒RNA的非自身特征以触发IFN反应。最近越来越多的证据表明，一些特定的非典型自身RNA也可独立于病毒感染而导致RLRs激活。重要的是，当这些RNA引起的RLR激活或错义突变导致的构象变化持续存在时，由此产生的I型IFN持续产生将导致自身免疫性疾病。在此，我们聚焦于由RLRs慢性激活引起的自身免疫性疾病，并讨论其发病的可能机制。
