Although transient activation of the cGAS-STING pathway is essential to trigger the host defense in response to pathogen invasion, chronic STING activation has been shown to be associated with several autoinflammatory diseases, such as Aicardi-Goutieres syndrome (AGS), systemic lupus erythematosus (SLE), and STING-associated vasculopathy with onset in infancy (SAVI) (12, –, 14), as well as inflammation-driven tumor genesis (15). Here, STING1 is linked to Aicardi-Goutieres syndrome.