Journal
NATURE REVIEWS IMMUNOLOGY
Volume 22, Issue 8, Pages 471-483Publisher
NATURE PORTFOLIO
DOI: 10.1038/s41577-021-00633-9
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Funding
- European Research Council (ERC) under the European Union's Horizon 2020 research and innovation programme [786142]
- French National Research Agency under the 'Investments for the Future' programme [ANR-10-IAHU-01]
- NIHR UK Rare Genetic Disease Research Consortium
- European Research Council (ERC) [786142] Funding Source: European Research Council (ERC)
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The term 'type I interferonopathy' was coined 10 years ago to describe rare genetic diseases caused by aberrant upregulation of type I interferon signaling. Viral nucleic acid detection is crucial for effective immune response, but failure in self versus non-self discrimination can lead to Mendelian inborn errors of immunity characterized by upregulation of type I interferon signaling.
The term 'type I interferonpathy' was coined 10 years ago to describe rare genetic diseases that are caused by an aberrant upregulation of type I interferon signalling. Here, Crow and Stetson discuss our current understanding of the type I interferonpathies, 10 years on. As brutally demonstrated by the COVID-19 pandemic, an effective immune system is essential for survival. Developed over evolutionary time, viral nucleic acid detection is a central pillar in the defensive armamentarium used to combat foreign microbial invasion. To ensure cellular homeostasis, such a strategy necessitates the efficient discrimination of pathogen-derived DNA and RNA from that of the host. In 2011, it was suggested that an upregulation of type I interferon signalling might serve as a defining feature of a novel set of Mendelian inborn errors of immunity, where antiviral sensors are triggered by host nucleic acids due to a failure of self versus non-self discrimination. These rare disorders have played a surprisingly significant role in informing our understanding of innate immunity and the relevance of type I interferon signalling for human health and disease. Here we consider what we have learned in this time, and how the field may develop in the future.
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