Aicardi-Goutières syndrome (AGS) is a monogenic inflammatory encephalopathy caused by mutations in any one of nine genes (TREX1, RNASEH2A/B/C, SAMHD1, ADAR1, IFIH1, LSM11, and RNU7-1) encoding for proteins involved in the metabolism and detection of nucleic acids (NAs; Aicardi and Goutieres, 1984; Crow et al., 2015b; Uggenti et al., 2020). Here, RNASEH2A is linked to Aicardi-Goutieres syndrome.