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Article

ADAR1 averts fatal type I interferon induction by ZBP1.

Nature - 1 Jul 2022

Jiao Huipeng, Wachsmuth Laurens, Wolf Simone, Lohmann Juliane, Nagata Masahiro, Kaya Göksu Gökberk, Oikonomou Nikos, Kondylis Vangelis, Rogg Manuel, Diebold Martin, Tröder Simon E, Zevnik Branko, Prinz Marco, Schell Christoph, Young George R, Kassiotis George, Pasparakis Manolis

Abstract excerpt

Mutations of the ADAR1 gene encoding an RNA deaminase cause severe diseases associated with chronic activation of type I interferon (IFN) responses, including Aicardi-Goutières syndrome and bilateral striatal necrosis1-3. The IFN-inducible p150 isoform of ADAR1 contains a Zα domain that recognizes RNA with an alternative left-handed double-helix structure, termed Z-RNA4,5. Hemizygous ADAR1 mutations in the Zα...

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Identifiers

PMID: 35859176
DOI: 10.1038/s41586-022-04878-9
PMCID: PMC9329096

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Related research

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ADAR1 averts fatal type I interferon induction by ZBP1.PMID 35859176Read ↗

ADAR1 mutation causes ZBP1-dependent immunopathology.Semantic proximity Read ↗

ADAR1 interaction with Z-RNA promotes editing of endogenous double-stranded RNA and prevents MDA5-dependent immune activation.Semantic proximity Read ↗

Disruption of Z-RNA–binding of ADAR1 induces Aicardi-Goutières syndrome–like encephalopathy in miceSemantic proximity Read ↗

Adenosine-to-inosine editing of endogenous Z-form RNA by the deaminase ADAR1 prevents spontaneous MAVS-dependent type I interferon responses.Semantic proximity Read ↗

Mutations in the adenosine deaminase ADAR1 that prevent endogenous Z-RNA binding induce Aicardi-Goutières-syndrome-like encephalopathy.Semantic proximity Read ↗

ADAR1 interaction with Z-RNA promotes editing of endogenous double-stranded RNA and prevents MDA5-dependent immune activationSemantic proximity Read ↗

Biological consequences of Z-nucleic acid sensing by ZBP1 and ADAR1.Semantic proximity Read ↗

ADAR1 Zα domain P195A mutation activates the MDA5-dependent RNA-sensing signaling pathway in brain without decreasing overall RNA editing.Semantic proximity Read ↗

The ADAR1 editome reveals drivers of editing-specificity for ADAR1-isoformsSemantic proximity Read ↗

ZBP1: A Powerful Innate Immune Sensor and Double-Edged Sword in Host ImmunitySemantic proximity Read ↗

Functions of the RNA Editing Enzyme ADAR1 and Their Relevance to Human DiseasesSemantic proximity Read ↗

ADAR1 and ZBP1 in innate immunity, cell death, and diseaseSemantic proximity Read ↗

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