Intracytoplasmic accumulation of dsDNA and ssDNA, as pathogen-associated molecular patterns, causes autoimmune reactions.62, 63, 64 In these diseases, Aicardi-Goutières syndrome is caused by the accumulation of a large amount of damaged DNA in the cytoplasm caused by TREX1 mutation, which strongly triggers the cGAS-STING pathway, resulting in systemic autoimmunity.65 Here, TREX1 is linked to Aicardi-Goutières syndrome.