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The\n                    <jats:italic>TREX1\u2010P212fs<\/jats:italic>\n                    mutation is thought to be linked to systemic lupus erythematosus (SLE). Here, we analyzed the functions of 20 TREX1 mutants and found that the TREX1\u2010P212fs mutant was able to reduce DNA enzyme activity and missed endoplasmic reticulum localization. Mouse\u2010derived in\u00a0situ\n                    <jats:italic>Trex1<\/jats:italic>\n                    models of transcoding mutations have not been reported. We successfully constructed\n                    <jats:italic>Trex1\u2010P212fs<\/jats:italic>\n                    mice by CRISPR\u2010Cas9 technology.\n                    <jats:italic>Trex1<\/jats:italic>\n                    <jats:sup>\n                      <jats:italic>P212fs\/P212fs<\/jats:italic>\n                    <\/jats:sup>\n                    mice exhibit systemic inflammation, lymphocytosis, vasculitis, and kidney disease. The excessive autoantibody production was also present in these mice. We further demonstrated that TREX1 (1\u2013212) protein lost its interaction with RPN1, the subunit that makes up the oligosaccharyl transferase (OST) complex. These data suggest an important role of TREX1\u2010C\u2010terminal association with the endoplasmic reticulum in inducing immune activation and the\n                    <jats:italic>Trex1\u2010P212fs<\/jats:italic>\n                    model may provide theoretical support for a better understanding of SLE treatment and defense.\n                  <\/jats:p>","DOI":"10.1096\/fj.202401579r","type":"journal-article","created":{"date-parts":[[2024,12,2]],"date-time":"2024-12-02T11:03:51Z","timestamp":1733137431000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["The\n                    <scp>SLE<\/scp>\n                    \u2010associated\n                    <i>TREX1\u2010P212fs<\/i>\n                    mutation disrupts\n                    <scp>ER<\/scp>\n                    association leading to type I interferonopathy"],"prefix":"10.1096","volume":"38","author":[{"ORCID":"https:\/\/orcid.org\/0009-0005-9568-3610","authenticated-orcid":false,"given":"Shan","family":"Xu","sequence":"first","affiliation":[{"name":"Fujian Key Laboratory of Innate Immune Biology, Biomedical Research Center of South China, College of Life Sciences Fujian Normal University  Fuzhou China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7153-8391","authenticated-orcid":false,"given":"Nanyang","family":"Xiao","sequence":"additional","affiliation":[{"name":"Fujian Key Laboratory of Innate Immune Biology, Biomedical Research Center of South China, College of Life Sciences Fujian Normal University  Fuzhou China"},{"name":"Department of Microbiology, Cummings Life Science Center University of Chicago  Chicago Illinois 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