To better investigate STING hyperactivation in vivo and to model SAVI disease, the variants N154S and V155M were introduced into mouse models using knock-in of the murine orthologs N153S and V154M.40,41 Similar to SAVI patients, these “SAVI” mice spontaneously develop a skin and lung disease and suffer premature death. Here, STING1 is linked to STING-associated vasculopathy with onset in infancy.