For example, in Aicardi-Goutières Syndrome (AGS), mutations in cytosolic exonuclease TREX1 lead to an accumulation of self-DNA in the cytosol that is sensed by cGAS to drive STING-dependent IFN-I production leading to a severe autoinflammatory disease (Crow et al., 2006; Gray et al., 2015; Gall et al., 2012; Gao et al., 2015). Here, STING1 is linked to Aicardi-Goutieres syndrome.