Journal
NATURE REVIEWS IMMUNOLOGY
Volume 15, Issue 7, Pages 429-440Publisher
NATURE PUBLISHING GROUP
DOI: 10.1038/nri3850
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Funding
- European Research Council [GA 309449]
- National Research Agency (France) under the Investments for the Future programme [ANR-10-IAHU-01]
- European FP7 Marie Curie Actions [268311]
- Laboratory of Excellence (LABEX) Vaccine Research Institute (VRI) [ANR-10-LABX-77]
- LABEX Dendritic Cell Biology (DCBIOL) [ANR-10-IDEX-0001-02 PSL, ANR-11-LABX-0043]
- European Research Council grant [309848 HIVINNATE]
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Dissection of the genetic basis of Aicardi-Goutieres syndrome has highlighted a fundamental link between nucleic acid metabolism, innate immune sensors and type I interferon induction. This had led to the concept of the human interferonopathies as a broader set of Mendelian disorders in which a constitutive upregulation of type I interferon activity directly relates to disease pathology. Here, we discuss the molecular and cellular basis of the interferonopathies, their categorization, future treatment strategies and the insights they provide into normal physiology.
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