Rivara Sophie, Ablasser Andrea
Global Health Institute, Swiss Federal Institute of Technology Lausanne, Lausanne, Switzerland.
J Exp Med. 2020 Nov 2;217(11). doi: 10.1084/jem.20201517.
Two studies published in this issue of JEM, by Lepelley et al. (https://doi.org/10.1084/jem.20200600) and Deng et al. (https://doi.org/10.1084/jem.20201045), and two additional manuscripts by Mukai et al. (https://doi.org/10.1101/2020.05.20.107664 Preprint v1) and Steiner et al. (https://doi.org/10.1101/2020.07.09.194399 Preprint v1) demonstrate that COPA syndrome-associated high interferon titers are linked to mutations in COPA preventing STING's retrieval from the Golgi back to the ER and thereby causing chronic immune activation.