Mutations in eight genes are associated with AGS, with a clustering of genes involved in cytosolic DNA metabolism (TREX1, RNASEH2B, RNASEH2C, RNASEH2A, SAMHD1) and those regulating cytosolic RNA metabolism (ADAR and IFIH1) [129, 131]. This evidence concerns the gene ADAR and Aicardi-Goutieres syndrome.