Associated Papers
Heterozygous ADAR mutant mice exhibit RNA sensing-dependent neuroinflammation and phenotypes associated with Aicardi-Goutières syndrome.
Guo X et al.
Iscience, 2026
Microgliosis and aberrant interferon response in Adar Mavs brain are rescued by PKR removal.
Lacovich V et al.
Brain : a Journal of Neurology, 2026
Endothelial ADAR1 Deficit Induces the NOCT-IRF7 Axis in Pulmonary Hypertension.
Woodcock CC et al.
Circulation Research, 2026
Biochemical profiling and structural basis of ADAR1-mediated RNA editing.
Deng X et al.
Molecular Cell, 2025
The prototypical interferonopathy: Aicardi-Goutières syndrome from bedside to bench.
Hofer MJ et al.
Immunological Reviews, 2024
Structural impacts of two disease-linked ADAR1 mutants: a molecular dynamics study.
Huang WC et al.
Journal of Computer-aided Molecular Design, 2024
Novel insights into double-stranded RNA-mediated immunopathology.
de Reuver R et al.
Nature Reviews. Immunology, 2024
Impact of Disease-Associated Mutations on the Deaminase Activity of ADAR1.
Karki A et al.
Biochemistry, 2024
ADAR1 Zα domain P195A mutation activates the MDA5-dependent RNA-sensing signaling pathway in brain without decreasing overall RNA editing.
Guo X et al.
Cell Reports, 2023
Innate immune activation without immune cell infiltration in brains of murine models of Aicardi-Goutières Syndrome.
Wiley CA et al.
Brain Pathology (zurich, Switzerland), 2023
The RNA-editing enzyme ADAR1: a regulatory hub that tunes multiple dsRNA-sensing pathways.
Nakahama T et al.
International Immunology, 2023
An AGS-associated mutation in ADAR1 catalytic domain results in early-onset and MDA5-dependent encephalopathy with IFN pathway activation in the brain.
Guo X et al.
Journal of Neuroinflammation, 2022
The role of RNA editing enzyme ADAR1 in human disease.
Song B et al.
Wiley Interdisciplinary Reviews. Rna, 2022
An Aicardi-Goutières Syndrome-Causative Point Mutation in Adar1 Gene Invokes Multiorgan Inflammation and Late-Onset Encephalopathy in Mice.
Inoue M et al.
Journal of Immunology (baltimore, Md. : 1950), 2021
Aicardi-Goutières syndrome-associated mutation at ADAR1 gene locus activates innate immune response in mouse brain.
Guo X et al.
Journal of Neuroinflammation, 2021
RNA Editing in Interferonopathies.
Frassinelli L et al.
Methods in Molecular Biology (clifton, N.j.), 2021
High-throughput mutagenesis reveals unique structural features of human ADAR1.
Park S et al.
Nature Communications, 2020
Adenosine-to-inosine RNA editing in the immune system: friend or foe?
Nakahama T et al.
Cellular and Molecular Life Sciences : Cmls, 2020
Sources of discordance among germ-line variant classifications in ClinVar.
Yang S et al.
Genetics in Medicine : Official Journal of the American College of Medical Genetics, 2017
Genetic, Phenotypic, and Interferon Biomarker Status in ADAR1-Related Neurological Disease.
Rice GI et al.
Neuropediatrics, 2017
Effects of Aicardi-Goutières syndrome mutations predicted from ADAR-RNA structures.
Fisher AJ et al.
Rna Biology, 2017
Global Carrier Rates of Rare Inherited Disorders Using Population Exome Sequences.
Fujikura K
Plos One, 2016
Characterization of human disease phenotypes associated with mutations in TREX1, RNASEH2A, RNASEH2B, RNASEH2C, SAMHD1, ADAR, and IFIH1.
Crow YJ et al.
American Journal of Medical Genetics. Part A, 2015
Mutations in ADAR1 cause Aicardi-Goutières syndrome associated with a type I interferon signature.
Rice GI et al.
Nature Genetics, 2012