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As such, the induction, transmission, and resolution of the type I interferon\u2013mediated immune response are tightly regulated. As defined, the type I interferonopathies represent discrete examples of a disturbance of the homeostatic control of this system caused by Mendelian mutations. Considering the complexity of the interferon response, the identification of further monogenic diseases belonging to this disease grouping seems likely, with the recognition of type I interferonopathies becoming of increasing clinical importance as treatment options are developed based on an understanding of disease pathology and innate immune signaling. Definition of the type I interferonopathies indicates that autoinflammation can be both interferon and noninterferon related, and that a primary disturbance of the innate immune system can \u201cspill over\u201d into autoimmunity in some cases. Indeed, that several non-Mendelian disorders, most particularly systemic lupus erythematosus and dermatomyositis, are also characterized by an up-regulation of type I interferon signaling suggests the possibility that insights derived from this work will have relevance to a broader field of clinical medicine.<\/jats:p>","DOI":"10.1084\/jem.20161596","type":"journal-article","created":{"date-parts":[[2016,11,7]],"date-time":"2016-11-07T15:07:38Z","timestamp":1478531258000},"page":"2527-2538","update-policy":"https:\/\/doi.org\/10.1084\/jem.crossmarkpolicy","source":"Crossref","is-referenced-by-count":434,"title":["Type I interferon\u2013mediated monogenic autoinflammation: The type I interferonopathies, a conceptual overview"],"prefix":"10.1084","volume":"213","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1300-0187","authenticated-orcid":false,"given":"Mathieu P.","family":"Rodero","sequence":"first","affiliation":[{"name":"INSERM UMR 1163, Laboratory of Neurogenetics and Neuroinflammation, 75015 Paris, France 1"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7211-7564","authenticated-orcid":false,"given":"Yanick J.","family":"Crow","sequence":"additional","affiliation":[{"name":"INSERM UMR 1163, Laboratory of Neurogenetics and Neuroinflammation, 75015 Paris, France 1"},{"name":"Paris Descartes University, Sorbonne-Paris-Cit\u00e9, Institut Imagine, H\u00f4pital Necker, 75015 Paris, France 2"},{"name":"Faculty of Biology, Medicine, and Health, Division of Evolution and Genomic Sciences, School of Biological Sciences, University of Manchester, Manchester M13 9NT, England, UK 3"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"291","published-online":{"date-parts":[[2016,11,7]]},"reference":[{"key":"2023072608545142200_bib1","doi-asserted-by":"publisher","first-page":"406","DOI":"10.1093\/rheumatology\/kes181","article-title":"Heterozygous TREX1 p.Asp18Asn mutation can cause variable neurological symptoms in a family with Aicardi-Gouti\u00e8res syndrome\/familial chilblain lupus","volume":"52","author":"Abe","year":"2013","journal-title":"Rheumatology (Oxford)."},{"key":"2023072608545142200_bib2","doi-asserted-by":"publisher","first-page":"4634","DOI":"10.4049\/jimmunol.1401337","article-title":"Intrinsic self-DNA triggers inflammatory disease dependent on STING","volume":"193","author":"Ahn","year":"2014","journal-title":"J. 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