These mutants of STING reveal constitutive activation of STING and hypersensitivity to cGAMP, resulting in phosphoralation of TBK1 and IRF3 to promote interferon-response genes or other genes expression that mediates inflammation in vascular endothelial cells, which is an important cause to lesional skin from SAVI patients. The gene discussed is STING1; the disease is STING-associated vasculopathy with onset in infancy.