Abstract

Some RNAs can assume a Z conformation, an unusual, left-handed turn. In this issue of Immunity, three studies report that mutations in the Zα-RNA binding domain of the adenosine deaminase ADAR1 are sufficient to induce autoinflammatory disease in mice, which models human Aicardí-Goutières syndrome, highlighting the important role of Z-RNA editing in limiting innate immune recognition of endogenous RNA.

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