RNASEH2A and Aicardi-Goutieres syndrome: Aicardi-Goutières syndrome is an inherited subacute encephalopathy caused by mutations in TREX1, RNASEH2B, RNASEH2C, RNASEH2A, SAMHD1, ADAR1, IFIH1. All these AGS-related genes are involved in the nucleic acid metabolism or sensing, promoting, in physiological conditions, both the removal or identification of the presence of foreign nucleic acids.