AGS involves recessive and dominant mutations in RNAses (RNASEH2A, RNASEH2B, RNASEH2C), a DNAse (TREX1), double-stranded RNA (dsRNA) editing (ADAR), dsRNA recognition and binding (IFIH1), and activation of the innate immune system (SAMHD1, IFIH1) (Crow et al. 2015). This evidence concerns the gene TREX1 and Aicardi-Goutieres syndrome.