The Type I Interferonopathies.
Type I interferons (IFNs) play a central role in the immune defense against viral infections. Type I IFN activation is induced by pattern-recognition receptors of the innate immune system that sense pathogen-derived nucleic acids. Cellular responses to type I IFN signaling are orchestrated by a complex network of regulatory pathways that involve both the innate and adaptive immune system. The genetic and molecular dissection of rare Mendelian disorders associated with constitutive overproduction of type I IFN has provided unique insight into cell-intrinsic disease mechanisms that initiate and sustain autoinflammation and autoimmunity and that are caused by disturbances in the intracellular nucleic acid metabolism or in cytosolic nucleic acid-sensing pathways. Collectively, these findings have greatly advanced our understanding of mechanisms that protect the organism against inappropriate immune activation triggered by self nucleic acids while maintaining a prompt and efficient immune response to foreign nucleic acids derived from invading pathogens.
Department of Pediatrics, Medizinische Fakultät Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany; email: minae.lee-kirsch@uniklinikum-dresden.de.
Min Ae
MA
Lee-Kirsch
D000818:Animals; D001327:Autoimmune Diseases / Q000235:genetics* / Q000276:immunology*; D020274:Autoimmune Diseases of the Nervous System / Q000235:genetics / Q000276:immunology; D006801:Humans; D007113:Immunity, Innate; D007249:Inflammation / Q000235:genetics* / Q000276:immunology*; D007370:Interferon Type I / Q000276:immunology*; D008180:Lupus Erythematosus, Systemic / Q000235:genetics / Q000276:immunology; D009421:Nervous System Malformations / Q000235:genetics / Q000276:immunology; D009696:Nucleic Acids / Q000276:immunology
D007370:Interferon Type I; D009696:Nucleic Acids
autoimmunity; autoinflammation; innate immunity; nucleic acid metabolism; nucleic acid sensing; type I interferon