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Article

ADAR1 mutation causes ZBP1-dependent immunopathology.

Nature - 1 Jul 2022

Hubbard Nicholas W, Ames Joshua M, Maurano Megan, Chu Lan H, Somfleth Kim Y, Gokhale Nandan S, Werner Margo, Snyder Jessica M, Lichauco Katrina, Savan Ram, Stetson Daniel B, Oberst Andrew

Abstract excerpt

The RNA-editing enzyme ADAR1 is essential for the suppression of innate immune activation and pathology caused by aberrant recognition of self-RNA, a role it carries out by disrupting the duplex structure of endogenous double-stranded RNA species1,2. A point mutation in the sequence encoding the Z-DNA-binding domain (ZBD) of ADAR1 is associated with severe autoinflammatory disease3-5. ZBP1 is the only other...

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Identifiers

PMID: 35859177
DOI: 10.1038/s41586-022-04896-7
PMCID: PMC9339495

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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