Since STING plays critical roles in innate immune responses to DNA pathogens and aberrant self DNA, dys-regulation of STING has been implicated in various autoimmune syndromes such as systemic lupus erythematosus (SLE), multiple sclerosis, Aicardi–Goutières syndrome and STING-associated vasculopathy with onset in infancy (SAVI)20–24. Here, STING1 is linked to Aicardi-Goutieres syndrome.