AGS is mainly inherited in an AR manner and it results in mutations of genes encoding for intracellular nucleic acids degradation enzymes: 3′-5′ DNA exonuclease -TREX1 (AGS1), ribonucleases as the ribonuclease H2 subunit B RNASEH2B (AGS2), ribonuclease H2 subunit C RNASEH2C (AGS3) and ribonuclease H2 subunit A RNASEH2A (AGS4), SAM and HD domain containing deoxynucleoside triphosphate triphosphohydrolase 1 SAMHD1 (AGS5) and Adenosine deaminase acting on RNA 1 ADAR1 (AGS6), causing inappropriate accumulation of endogenous nucleic acids [119,125,130,131,132,133,134,135,136]. Here, RNASEH2A is linked to Aicardi-Goutieres syndrome.