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Article

Microgliosis and aberrant interferon response in Adar Mavs brain are rescued by PKR removal.

Brain : a journal of neurology - 7 Feb 2026

Lacovich Valentina, Stejskal Stanislav, Locker Kovačovicova Kristina, Potěšil David, Pustka Vaclav, Vukić Dragana, Texlova Katerina, Linhartova Pavla, Melicherova Janka, Sinigaglia Ketty, Havas Daniel, Keegan Liam P, O'Connell Mary Anne

Abstract excerpt

Mutations in the human ADAR gene, encoding adenosine deaminase acting on RNA 1 (ADAR1), cause Aicardi-Goutières syndrome 6, which is a severe auto-inflammatory encephalopathy with aberrant interferon (IFN) induction. AdarΔ2-13 null mutant mouse embryos lacking ADAR1 protein die with high levels of IFN-stimulated gene (ISG) transcripts. In Adar Mavs double mutants also lacking the Mitochondrial antiviral...

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Share to Adaptor Proteins, Signal Transducing
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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Source

Identifiers

PMID: 40920008
DOI: 10.1093/brain/awaf315

Genes

ADAR
MAVS

Variants

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 8 Sept 2026.

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Microgliosis and aberrant interferon response in Adar Mavs brain are rescued by PKR removal.PMID 40920008Read ↗

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Aicardi-Goutières syndrome-associated mutation at ADAR1 gene locus activates innate immune response in mouse brain.Semantic proximity Read ↗

PKR and the Integrated Stress Response drive immunopathology caused by ADAR1 mutationSemantic proximity Read ↗

An internal deletion of ADAR rescued by MAVS deficiency leads to a minute phenotype.Semantic proximity Read ↗

Aicardi-Goutières Syndrome Associated <i>ADAR</i> G1007R mutation dominantly induces neuroinflammation in mouse brainSemantic proximity Read ↗

Protein kinase R and the integrated stress response drive immunopathology caused by mutations in the RNA deaminase ADAR1.Semantic proximity Read ↗

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Aberrant activation of the innate immune sensor PKR by self dsRNA is prevented by direct interaction with ADAR1Semantic proximity Read ↗

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