In two autoinflammatory monogenic diseases, STING-associated vasculopathy with onset in infancy (SAVI) (Liu et al., 2014) and the COPA syndrome (Watkin et al., 2015), STING was constitutively active without DNA stimulation and localized not to the ER but to the perinuclear compartments including the Golgi (Jeremiah et al., 2014; Mukai et al., 2016; Ogawa et al., 2018; Lepelley et al., 2020; Deng et al., 2020; Mukai et al., 2021; Taguchi et al., 2021). This evidence concerns the gene STING1 and STING-associated vasculopathy with onset in infancy.