AGS is caused by mutations in genes (SAMHD1, ADAR1, TREX1, IFIH1, RNASEH2A, RNASEH2B, RNASEH2C, LSM11, and RNU7-1) many of which lead to accumulation of DNA in the cytosol that activates cGAS signaling (143). This evidence concerns the gene CGAS and Aicardi-Goutieres syndrome.