STING1 and Aicardi-Goutieres syndrome: Moreover, mutations in the DNase enzyme three prime exonuclease 1 (TREX1) were associated with the development of autoinflammatory conditions such as Aicardi–Goutieres syndrome (AGS) and SLE, culminating in autoinflammation-mediated lethality, which is dependent on aberrant STING pathway activation as a result of the inability of the cells to clear DNA from dying cells (13, 14).