Kato Hiroki, Fujita Takashi
Laboratory of Molecular Genetics, Institute for Virus Research, and Laboratory of Molecular and Cellular Immunology, Graduate School of Biostudies, Kyoto University, Kyoto, Japan; PRESTO, Science and Technology Agency (JST), Kawaguchi, Saitama 332-0012, Japan.
Laboratory of Molecular Genetics, Institute for Virus Research, and Laboratory of Molecular and Cellular Immunology, Graduate School of Biostudies, Kyoto University, Kyoto, Japan.
Curr Opin Immunol. 2015 Dec;37:40-5. doi: 10.1016/j.coi.2015.10.002.
Type I interferon (IFN) plays an essential role in antiviral innate immune responses and also in adaptive immune responses. Defects in the production of IFN markedly increase susceptibility to viral invasion and attenuate the acquired immunity. Recently an increased expression of type I IFN, also termed IFN signature, has been reported in patients with autoimmune diseases such as systemic lupus erythematosus (SLE) and Aicardi-Goutières syndrome (AGS). The evidence clearly shows that the initiation and termination of IFN production should be tightly controlled. RIG-I-like receptors (RLRs) are viral RNA sensors and are essential for type I IFN induction. We herein summarize recent reports on RLR mutations in patients and MDA5 mutant mice, and discuss possible mechanisms by which aberrant activation of RLRs can cause autoimmunity.
I型干扰素（IFN）在抗病毒固有免疫反应以及适应性免疫反应中发挥着至关重要的作用。IFN产生缺陷会显著增加对病毒侵袭的易感性，并削弱获得性免疫。最近，在系统性红斑狼疮（SLE）和Aicardi-Goutières综合征（AGS）等自身免疫性疾病患者中，已报道I型干扰素表达增加，也称为IFN特征。证据清楚地表明，IFN产生的起始和终止应受到严格控制。RIG-I样受体（RLRs）是病毒RNA传感器，对I型干扰素的诱导至关重要。我们在此总结了关于患者和MDA5突变小鼠中RLR突变的最新报道，并讨论了RLRs异常激活可能导致自身免疫的潜在机制。
