title: RIG-I-like receptors and autoimmune diseases.
abstract: 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.
authors.affiliation: 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.
authors.forename: Hiroki
authors.initials: H
authors.lastname: Kato
authors.affiliation: Laboratory of Molecular Genetics, Institute for Virus Research, and Laboratory of Molecular and Cellular Immunology, Graduate School of Biostudies, Kyoto University, Kyoto, Japan. Electronic address: tfujita@virus.kyoto-u.ac.jp.
authors.forename: Takashi
authors.initials: T
authors.lastname: Fujita
mesh_terms: D000818:Animals; D001327:Autoimmune Diseases / Q000276:immunology*; D000071457:DEAD Box Protein 58; D053487:DEAD-box RNA Helicases / Q000235:genetics / Q000276:immunology / Q000378:metabolism*; D006801:Humans; D007370:Interferon Type I / Q000235:genetics / Q000378:metabolism*; D000072640:Interferon-Induced Helicase, IFIH1; D051379:Mice; D008817:Mice, Mutant Strains; D009154:Mutation / Q000235:genetics; D012367:RNA, Viral / Q000378:metabolism; D011971:Receptors, Immunologic; D059467:Transcriptome
chemical_list: D007370:Interferon Type I; D012367:RNA, Viral; D011971:Receptors, Immunologic; C452820:RIGI protein, human; C510592:Ifih1 protein, mouse; D000071457:DEAD Box Protein 58; D053487:DEAD-box RNA Helicases; D000072640:Interferon-Induced Helicase, IFIH1