Recognition of cytosolic foreign DNA by cGAS is critical for triggering an innate immune reaction in the host and for combating infections with various microbes, including DNA viruses, retroviruses, and some bacterial pathogens.3,35,36 However, inappropriate chronic activation of cGAS by endogenous DNA contributes to severe autoimmune diseases, such as lupus and AGS.11,13 Thus, the activity and expression of cGAS must be tightly regulated to ensure immune homeostasis. Here, CGAS is linked to Aicardi-Goutieres syndrome.