In vivo, Adar mutant Drosophila and Adar1 mouse AGS models have been used to show that dysfunctional ADAR1 results in innate immune activation in brain-like tissue and mammalian brains, respectively (Deng et al., 2020; Guo et al., 2021; Inoue et al., 2021). This evidence concerns the gene ADAR and Aicardi-Goutieres syndrome.