Over the last decade, chronic activation or overactivation of the cGAS-STING pathway has been identified to drive a large fraction of monogenic disorders that were grouped together under the term type I interferonopathies.626 This work was largely pioneered by work on the molecular mechanisms underlying Aicardi Goutières syndrome (AGS), a rare autoimmune encephalopathy. The gene discussed is STING1; the disease is Aicardi-Goutieres syndrome.