Notably, mouse models of AGS, resulting from biallelic TREX1-deficiency, are protected from lethality by genetic deletion of cGAS, STING, or the type I interferon receptor (IFNAR1) (Gall et al., 2012; Gao et al., 2015; Stetson et al., 2008; Gray et al., 2015). Here, CGAS is linked to Aicardi-Goutieres syndrome.