Although STING1-mediated innate immunity plays significant roles in shaping host defense against microbe invasion and tumor growth (5), aberrative activation of STING1 can also disturb immune balance, thereby leading to pathological conditions and human diseases, such as STING-associated vasculopathy with onset in infancy (SAVI), Aicardi-Goutieres syndrome, systemic lupus erythematosus (SLE), and sepsis (6, 7), as well as neurodegenerative diseases (8), and metabolic diseases (9). This evidence concerns the gene STING1 and Aicardi-Goutieres syndrome.