Mutations in other DNA-related enzymes, such as ssDNA 3′–5′ exonuclease TREX1 (three prime repair exonuclease 1; DNASEIII), dsRNA-editing enzyme ADAR1 (adenosine deaminase acting on RNA 1), and dNTP triphosphohydrolase SAMHD1 (SAM and HD domain containing deoxynucleoside triphosphate triphosphohydrolase 1), have also been associated with AGS.171. Here, SAMHD1 is linked to Aicardi-Goutieres syndrome.