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 …

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 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 …

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 …

[PDF][PDF] 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 …

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 …

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 …

ADAR1 masks the cancer immunotherapeutic promise of ZBP1-driven necroptosis

T Zhang, C Yin, A Fedorov, L Qiao, H Bao… - Nature, 2022 - nature.com
Only a small proportion of patients with cancer show lasting responses to immune
checkpoint blockade (ICB)-based monotherapies. The RNA-editing enzyme ADAR1 is an …

Adar RNA editing-dependent and -independent effects are required for brain and innate immune functions in Drosophila

P Deng, A Khan, D Jacobson, N Sambrani… - Nature …, 2020 - nature.com
ADAR RNA editing enzymes are high-affinity dsRNA-binding proteins that deaminate
adenosines to inosines in pre-mRNA hairpins and also exert editing-independent effects …

ADAR1 and ZBP1 in innate immunity, cell death, and disease

R Karki, TD Kanneganti - Trends in immunology, 2023 - cell.com
ADAR1 and ZBP1 are the only two mammalian proteins that contain Zα domains, which are
thought to bind to nucleic acids in the Z-conformation. These two molecules are crucial in …