The SAVI model we have generated, based on the activity of a mutant human STING protein, represents not only a model system for dissecting mechanisms involved in the pathogenesis of SAVI but will also serve as a useful preclinical tool for the in vivo evaluation of therapeutics aimed at curtailing abnormal STING activity. This evidence concerns the gene STING1 and STING-associated vasculopathy with onset in infancy.