{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"institution":[{"name":"bioRxiv"}],"indexed":{"date-parts":[[2026,10,11]],"date-time":"2026-10-11T13:20:40Z","timestamp":1791724840550,"version":"4.3.4"},"posted":{"date-parts":[[2024,4,2]]},"group-title":"Molecular Biology","reference-count":28,"publisher":"openRxiv","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"accepted":{"date-parts":[[2024,4,2]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                <jats:p>Interferon-stimulated genes (ISGs) comprise a program of immune effectors important for host immune defense. When uncontrolled, ISGs play a central role in interferonopathies and other inflammatory diseases. The mechanisms responsible for turning on ISGs are not completely known. By investigating MATRIN3 (MATR3), a nuclear RNA-binding protein mutated in familial ALS, we found that perturbing MATR3 results in elevated expression of ISGs. Using an integrative approach, we elucidate a pathway that leads to activation of cGAS-STING. This outlines a plausible mechanism for pathogenesis in a subset of ALS, and suggests new diagnostic and therapeutic approaches for this fatal disease.<\/jats:p>\n                <jats:sec>\n                  <jats:title>One-Sentence Summary<\/jats:title>\n                  <jats:p>\n                    Mis-splicing of\n                    <jats:italic>Tudor Domain Containing 3<\/jats:italic>\n                    (\n                    <jats:italic>TDRD3<\/jats:italic>\n                    ) in the absence of MATR3 unleashes R-loops and interferon-stimulated genes.\n                  <\/jats:p>\n                <\/jats:sec>","DOI":"10.1101\/2024.04.01.587645","type":"posted-content","created":{"date-parts":[[2024,4,2]],"date-time":"2024-04-02T17:25:32Z","timestamp":1712078732000},"source":"Crossref","is-referenced-by-count":6,"title":["<i>MATRIN3<\/i>\n                  deficiency triggers autoinflammation via cGAS-STING activation"],"prefix":"10.64898","author":[{"given":"Zohirul","family":"Islam","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ahsan","family":"Polash","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masataka","family":"Suzawa","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bryan","family":"Chim","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Skyler","family":"Kuhn","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sabrina","family":"Sultana","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicholas","family":"Cutrona","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patrick 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