Walking a tightrope: the complex balancing act of R-loops in genome stability

JR Brickner, JL Garzon, KA Cimprich - Molecular cell, 2022 - cell.com
Although transcription is an essential cellular process, it is paradoxically also a well-
recognized cause of genomic instability. R-loops, non-B DNA structures formed when …

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 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 and its implications in cancer development and treatment

AR Baker, FJ Slack - Trends in Genetics, 2022 - cell.com
The family of adenosine deaminases acting on RNA (ADARs) regulates global gene
expression output by catalyzing adenosine-to-inosine (A-to-I) editing of double-stranded …

DAMP sensing and sterile inflammation: intracellular, intercellular and inter-organ pathways

Y Huang, W Jiang, R Zhou - Nature Reviews Immunology, 2024 - nature.com
Damage-associated molecular patterns (DAMPs) are endogenous molecules that are
released from host cells as a result of cell death or damage. The release of DAMPs in …

[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 …

The phenotype of the most common human ADAR1p150 Zα mutation P193A in mice is partially penetrant

Z Liang, AM Chalk, S Taylor, A Goradia… - EMBO …, 2023 - embopress.org
Abstract ADAR1‐mediated A‐to‐I RNA editing is a self‐/non‐self‐discrimination mechanism
for cellular double‐stranded RNAs. ADAR mutations are one cause of Aicardi–Goutières …

Endogenous retroelements as alarms for disruptions to cellular homeostasis

HT Lindholm, R Chen, DD De Carvalho - Trends in Cancer, 2023 - cell.com
Endogenous retroelements are DNA sequences which can duplicate and move to new
locations in the genome. Actively moving endogenous retroelements can be disruptive to the …

An AGS-associated mutation in ADAR1 catalytic domain results in early-onset and MDA5-dependent encephalopathy with IFN pathway activation in the brain

X Guo, RA Steinman, Y Sheng, G Cao… - Journal of …, 2022 - Springer
Abstract Background Aicardi–Goutières syndrome (AGS) is a severe neurodegenerative
disease with clinical features of early-onset encephalopathy and progressive loss of …

Impact of disease-associated mutations on the deaminase activity of ADAR1

A Karki, KB Campbell, S Mozumder, AJ Fisher… - Biochemistry, 2024 - ACS Publications
The innate immune system relies on molecular sensors to detect distinctive molecular
patterns, including viral double-stranded RNA (dsRNA), which triggers responses resulting …