Our findings on the essential role of LGP2 in type I IFN induction caused by ADAR1 dysfunction are strengthened by a recent report in which AdarP195A/p150− mice, which bear a mutation in the Zα domain of ADAR1 p150 (P195A) paired with a null allele of Adar (mimicking the most common ADAR mutation in AGS at P193), were intercrossed with various knockout models, including Dhx58−/− mice (Maurano et al, 2021). This evidence concerns the gene DHX58 and Aicardi-Goutieres syndrome.