Mechanistic dissection of IFN induction has instead been possible using expression of disease‐associated alleles in mice (Mackenzie et al, 2016; Pokatayev et al, 2016), but surprisingly, none of the mutant RNase H2‐expressing mice showed symptoms in their brains (Mackenzie et al, 2016), despite the fact that the brain is the organ most heavily affected in AGS and that patients display increased IFN activity in the cerebrospinal fluid (Crow et al, 2015). This evidence concerns the gene IFNA1 and Aicardi-Goutieres syndrome.