The earliest discovered cases of ligand-independent STING activation are gain-of-function (GOF) STING1 (also known as TMEM173) mutations in a rare autoinflammatory disease named STING-associated vasculopathy with onset in infancy (SAVI) (Jeremiah et al., 2014; Liu et al., 2014). The gene discussed is STING1; the disease is STING-associated vasculopathy with onset in infancy.