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Article

Reconfigured immunity in Adar heterozygous and Adar Mavs Eif2ak2 (PKR) triple mutant mice.

RNA (New York, N.Y.) - 16 Jul 2026

Linhartová Pavla, Quin Jaclyn E, Loja Tomáš, Melicherová Janka, Du Qiupei, Cherian Anna, Vukić Dragana, Modrý David, Tomandlová Marie, Červená Barbora, Stříbrná Helena, Östlund Farrants Ann-Kristin, Knight Andrea, Keegan Liam P, O'Connell Mary A

Abstract excerpt

Adenosine deaminase acting on RNA 1 (ADAR1) deaminates adenosine to inosine in dsRNA. ADAR1 RNA editing marks cellular dsRNA as self, preventing aberrant activation of the antiviral dsRNA sensor MDA5. Adar Ifih1 (MDA5) or Adar Mavs double mutant pups escape Adar mutant aberrant interferon induction but die early. Here, we show that long-lived Adar Mavs Eif2ak2 (PKR) mice display a new residual defect: failure to...

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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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Identifiers

PMID: 42103444
DOI: 10.1261/rna.081079.126

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

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Reconfigured immunity in Adar heterozygous and Adar Mavs Eif2ak2 (PKR) triple mutant mice.PMID 42103444Read ↗

Protein recoding by ADAR1-mediated RNA editing is not essential for normal development and homeostasis.Semantic proximity Read ↗

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

ADAR1 haploinsufficiency and sustained viral RdRp dsRNA synthesis synergize to dysregulate RNA editing and cause multi-system interferonopathySemantic 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 ↗

Generation of a new <i> Adar1p150 <sup>-/-</sup> </i> mouse demonstrates isoform-specific roles in embryonic development and adult homeostasisSemantic proximity Read ↗

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

ADAR1, inosine and the immune sensing system: distinguishing self from non-self.Semantic proximity Read ↗

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

The majority of A-to-I RNA editing is not required for mammalian homeostasisSemantic proximity Read ↗

RNA editing by ADAR1 prevents MDA5 sensing of endogenous dsRNA as nonself.Semantic proximity Read ↗

ADAR RNA editing in human disease; more to it than meets the I.Semantic proximity Read ↗

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