Intriguingly, this finding is corroborated in a recently described mouse model of COPA syndrome (CopaE241K/+ mice)27: CopaE241K/+ mice exhibit spontaneous activation of STING with upregulation of type I interferon signaling and systemic inflammation, all of which is abrogated in STING-deficient animals28. Here, STING1 is linked to autoimmune interstitial lung disease-arthritis syndrome.