We generated HAQ/SAVI(N153S) and AQ/SAVI(N153S) mice and found that the HAQ, AQ alleles prevent CD4 T cellpenia, increasing/restoring T-regs and alleviating/stopping tissue inflammation in SAVI mice, thus providing evidence for the in vivo significance of type I IFNs-independent, STING1-mediated cell death and potential AQ-based curative therapy for SAVI patients. The gene discussed is STING1; the disease is STING-associated vasculopathy with onset in infancy.