ADAR1 prevents autoinflammation by suppressing spontaneous ZBP1 activation

R de Reuver, S Verdonck, E Dierick, J Nemegeer… - Nature, 2022 - nature.com
The RNA-editing enzyme adenosine deaminase acting on RNA 1 (ADAR1) limits the
accumulation of endogenous immunostimulatory double-stranded RNA (dsRNA). In …

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 …

[HTML][HTML] Protein kinase R and the integrated stress response drive immunopathology caused by mutations in the RNA deaminase ADAR1

M Maurano, JM Snyder, C Connelly, J Henao-Mejia… - Immunity, 2021 - cell.com
The RNA deaminase ADAR1 is an essential negative regulator of the RNA sensor MDA5,
and loss of ADAR1 function triggers inappropriate activation of MDA5 by self-RNAs …

Mutations in ADAR1 cause Aicardi-Goutières syndrome associated with a type I interferon signature

GI Rice, PR Kasher, GMA Forte, NM Mannion… - Nature …, 2012 - nature.com
Adenosine deaminases acting on RNA (ADARs) catalyze the hydrolytic deamination of
adenosine to inosine in double-stranded RNA (dsRNA) and thereby potentially alter the …

The RNA-editing enzyme ADAR1 controls innate immune responses to RNA

NM Mannion, SM Greenwood, R Young, S Cox… - Cell reports, 2014 - cell.com
The ADAR RNA-editing enzymes deaminate adenosine bases to inosines in cellular RNAs.
Aberrant interferon expression occurs in patients in whom ADAR1 mutations cause Aicardi …

ADAR RNA editing in human disease; more to it than meets the I

A Gallo, D Vukic, D Michalík, MA O'connell, LP Keegan - Human genetics, 2017 - Springer
We review the structures and functions of ADARs and their involvements in human diseases.
ADAR1 is widely expressed, particularly in the myeloid component of the blood system, and …

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) …

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 …

ADAR1 averts fatal type I interferon induction by ZBP1

H Jiao, L Wachsmuth, S Wolf, J Lohmann, M Nagata… - Nature, 2022 - nature.com
Mutations of the ADAR1 gene encoding an RNA deaminase cause severe diseases
associated with chronic activation of type I interferon (IFN) responses, including Aicardi …

Ribonuclease L mediates the cell-lethal phenotype of double-stranded RNA editing enzyme ADAR1 deficiency in a human cell line

Y Li, S Banerjee, SA Goldstein, B Dong, C Gaughan… - Elife, 2017 - elifesciences.org
ADAR1 isoforms are adenosine deaminases that edit and destabilize double-stranded RNA
reducing its immunostimulatory activities. Mutation of ADAR1 leads to a severe …