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Mol. Sci.","IJMS"],"abstract":"<jats:p>Sj\u00f6gren\u2019s disease (SjD) is a systemic autoimmune disease characterized by exocrine dysfunction, lymphocytic infiltration of the exocrine glands, and prominent activation of the interferon (IFN) pathway. Although IFN signatures are recognized as a central feature of SjD, endogenous triggers that sustain chronic IFN-driven inflammation remain incompletely understood. Mitochondrial RNAs (mtRNAs), particularly double-stranded species, have recently emerged as immunostimulatory molecules capable of linking mitochondrial stress to innate immune activation. In this review, we discuss the biological basis of mtRNA biogenesis and processing, the formation and mislocalization of mitochondrial double-stranded RNAs, and their recognition by innate immune sensors relevant to induction of type I IFN. We also describe how epithelial stress, mitochondrial dysfunction, and mtRNA accumulation amplify IFN-rich inflammatory circuits and glandular injury in SjD. Experimental studies in salivary gland epithelial models and SjD-relevant tissues support a mechanistic role for the mtRNA\u2013type I IFN axis, while emerging clinical data suggest that extracellular mtRNA levels in saliva and plasma may have potential as biomarkers of disease activity and patient stratification. Although current evidence remains limited, the mtRNA\u2013type I IFN axis provides a biologically plausible link between epithelial stress and immune dysregulation, and may have translational implications in SjD.<\/jats:p>","DOI":"10.3390\/ijms27167295","type":"journal-article","created":{"date-parts":[[2026,8,17]],"date-time":"2026-08-17T12:01:26Z","timestamp":1786968086000},"page":"7295","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Mitochondrial RNA\u2013Type I Interferon Axis in Sj\u00f6gren\u2019s Disease: Molecular Mechanisms and Translational Implications"],"prefix":"10.3390","volume":"27","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6107-9523","authenticated-orcid":false,"given":"You-Jung","family":"Ha","sequence":"first","affiliation":[{"name":"Division of Rheumatology, Department of Internal Medicine, Seoul National University Bundang Hospital, Seongnam 13620, Republic of Korea"},{"name":"Department of Internal Medicine, Seoul National University College of Medicine, Seoul 03080, Republic of Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yeon Bi","family":"Han","sequence":"additional","affiliation":[{"name":"Department of Pathology and Translational Medicine, Seoul National University Bundang Hospital, Seongnam 13620, Republic of Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5986-4810","authenticated-orcid":false,"given":"Keun-Suh","family":"Kim","sequence":"additional","affiliation":[{"name":"Department of Periodontology, Section of Dentistry, Seoul National University Bundang Hospital, Seongnam 13620, Republic of Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8577-6964","authenticated-orcid":false,"given":"Woo-Jin","family":"Jeong","sequence":"additional","affiliation":[{"name":"Department of Otorhinolaryngology-Head & Neck Surgery, Seoul National University Bundang Hospital, Seongnam 13620, Republic of Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joon Young","family":"Hyon","sequence":"additional","affiliation":[{"name":"Department of Ophthalmology, Seoul National University Bundang Hospital, Seongnam 13620, Republic of Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3064-4643","authenticated-orcid":false,"given":"Yoosik","family":"Kim","sequence":"additional","affiliation":[{"name":"Center for RNA Research, Institute for Basic Science, Seoul 08826, Republic of Korea"},{"name":"Department of Chemical and Biomolecular Engineering, Korea Advanced Institute of Science and Technology, Daejeon 34141, Republic of Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7615-8611","authenticated-orcid":false,"given":"Yun Jong","family":"Lee","sequence":"additional","affiliation":[{"name":"Division of Rheumatology, Department of Internal Medicine, Seoul National University Bundang Hospital, Seongnam 13620, Republic of Korea"},{"name":"Department of Medical Device Development, Seoul National University College of Medicine, Seoul 03080, Republic of Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2026,8,15]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"102703","DOI":"10.1016\/j.coi.2025.102703","article-title":"Interferons in Sjogren\u2019s disease: Current status and future prospects","volume":"98","author":"Raftopoulou","year":"2026","journal-title":"Curr. 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