ADAR1 edits the SenZ and SenZ-ability of RNA

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

Automated summary

These studies indicate that mutations in the Za-RNA binding domain of ADAR1 may lead to autoinflammatory disease in mice, modeling human Aicardi-Goutie` res syndrome. This highlights the important role of Z-RNA editing in limiting innate immune recognition of endogenous RNA.