The type I interferonopathies: 10 years on

YJ Crow, DB Stetson - Nature Reviews Immunology, 2022 - nature.com
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 …

Novel insights into double-stranded RNA-mediated immunopathology

R de Reuver, J Maelfait - Nature Reviews Immunology, 2024 - nature.com
Recent progress in human and mouse genetics has transformed our understanding of the
molecular mechanisms by which recognition of self double-stranded RNA (self-dsRNA) …

ADAR1 mutation causes ZBP1-dependent immunopathology

NW Hubbard, JM Ames, M Maurano, LH Chu… - Nature, 2022 - nature.com
The RNA-editing enzyme ADAR1 is essential for the suppression of innate immune
activation and pathology caused by aberrant recognition of self-RNA, a role it carries out by …

Human ADAR1 prevents endogenous RNA from triggering translational shutdown

H Chung, JJA Calis, X Wu, T Sun, Y Yu, SL Sarbanes… - Cell, 2018 - cell.com
Type I interferon (IFN) is produced when host sensors detect foreign nucleic acids, but how
sensors differentiate self from nonself nucleic acids, such as double-stranded RNA (dsRNA) …

The role of RNA editing enzyme ADAR1 in human disease

B Song, Y Shiromoto, M Minakuchi… - Wiley Interdisciplinary …, 2022 - Wiley Online Library
Adenosine deaminase acting on RNA (ADAR) catalyzes the posttranscriptional conversion
of adenosine to inosine in double‐stranded RNA (dsRNA), which can lead to the creation of …

ADAR1p150 prevents MDA5 and PKR activation via distinct mechanisms to avert fatal autoinflammation

SB Hu, J Heraud-Farlow, T Sun, Z Liang, A Goradia… - Molecular Cell, 2023 - cell.com
Effective immunity requires the innate immune system to distinguish foreign nucleic acids
from cellular ones. Cellular double-stranded RNAs (dsRNAs) are edited by the RNA-editing …

Rewriting the transcriptome: adenosine-to-inosine RNA editing by ADARs

CR Walkley, JB Li - Genome biology, 2017 - Springer
One of the most prevalent forms of post-transcritpional RNA modification is the conversion of
adenosine nucleosides to inosine (A-to-I), mediated by the ADAR family of enzymes. The …

ADAR1 interaction with Z-RNA promotes editing of endogenous double-stranded RNA and prevents MDA5-dependent immune activation

R de Reuver, E Dierick, B Wiernicki, K Staes, L Seys… - Cell reports, 2021 - cell.com
Loss of function of adenosine deaminase acting on double-stranded RNA (dsRNA)-1
(ADAR1) causes the severe autoinflammatory disease Aicardi-Goutières syndrome (AGS) …

The majority of A-to-I RNA editing is not required for mammalian homeostasis

AM Chalk, S Taylor, JE Heraud-Farlow, CR Walkley - Genome biology, 2019 - Springer
Background Adenosine-to-inosine (A-to-I) RNA editing, mediated by ADAR1 and ADAR2,
occurs at tens of thousands to millions of sites across mammalian transcriptomes. A-to-I …

[HTML][HTML] ADAR1 Zα domain P195A mutation activates the MDA5-dependent RNA-sensing signaling pathway in brain without decreasing overall RNA editing

X Guo, S Liu, Y Sheng, M Zenati, T Billiar, A Herbert… - Cell reports, 2023 - cell.com
Variants of the RNA-editing enzyme ADAR1 cause Aicardi-Goutières syndrome (AGS), in
which severe inflammation occurs in the brain due to innate immune activation. Here, we …