Genes with disease-causing mutations in AGS include the repair exonuclease TREX1 (3’ repair exonuclease 1); RNASEH2 (ribonuclease H2) subunits A, B and C; the Sterile Alpha Motif (SAM) domain and Histidine-Aspartate (HD) domain-containing protein 1 (SAMHD1); the double-stranded RNA-specific adenosine deaminase enzyme ADAR1; and the double-stranded RNA sensor Melanoma Differentiation-Associated Protein 5 (MDA5), encoded by Interferon Induced with Helicase C Domain 1 (IFIH1) [94, 95]. Here, TREX1 is linked to Aicardi-Goutieres syndrome.