TREX1 and Aicardi-Goutieres syndrome: To study AGS pathogenesis, many cellular and animal models were developed (Figure 5), including several null mice for the genes involved in AGS (KO), mice expressing the human mutations (KIN) (TREX1 KO and KIN, RNASEH2A/B/C KO, RNASEH2A/B KIN, SAMHD1 KO, ADAR1 KO, IFIH1 KIN) and some zebrafish models (SAMDH1 and ADAR1 KO).