Mutant STING in SAVI patients leads to increased transcription of the type 1 IFN gene, resulting in JAK activation and phosphorylation of the STAT pathway, which further upregulates the transcription of IFN-responsive genes.[3] Seventeen mutation sites (N154S, V155M, V147L, V147M, S102P, F209L, F153V, R281Q, R284G, G166E, R284S, G207E, R281W, H72N, G158A, C206Y, F279L) have been reported in the STING gene, with p.V155M being the most prevalent.[11,12]. The gene discussed is SOAT1; the disease is STING-associated vasculopathy with onset in infancy.