Most patients with TREX1-associated AGS have very limited voluntary gross motor control and ability to communicate (Figure 2) (Adang et al., 2020) All genes associated with AGS have functions in nucleic acid sensing or nucleotide metabolism, suggesting that defects in these processes are responsible for the aberrant type I interferon activity in the disease (Liu and Ying, 2023). Here, TREX1 is linked to Aicardi-Goutieres syndrome.