{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T17:12:19Z","timestamp":1790874739772,"version":"4.1.0"},"reference-count":26,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2021,2,12]],"date-time":"2021-02-12T00:00:00Z","timestamp":1613088000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Ann Clin Transl Neurol"],"published-print":{"date-parts":[[2021,3]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Objective<\/jats:title><jats:p>This study aimed to investigate mitochondrial changes and the mitochondrial antiviral\u2010signaling protein (MAVS)\u2010type I interferon (IFN1) signaling pathway in the muscles of anti\u2010melanoma differentiation gene 5(MDA5) dermatomyositis (DM) patients.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Eleven anti\u2010MDA5 DM and ten antibody\u2010negative DM patients were included. Muscle biopsies were performed in all patients. Muscle pathology and mitochondrial morphology in particular were compared between two groups. The expression of MDA5, MAVS, interferon (IFN) regulatory factor 7, and IFN\u2010stimulated gene 15, which are components of the MAVS\u2010IFN1 signaling pathway, was measured in muscle specimen. The correlation between MAVS expression in muscles and disease phenotypes and muscle pathology were analyzed.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Anti\u2010MDA5 DM showed a significantly lower incidence of the characteristic DM pathology (<jats:italic>P<\/jats:italic>\u00a0&lt;\u00a00.05) than antibody\u2010negative DM, including perifascicular fiber atrophy, inflammation, and vasculopathy. Mitochondrial abnormalities in anti\u2010MDA5 patients revealed a high incidence of (8\/11,72.7%) and different pattern from that in antibody\u2010negative DM. MDA5, MAVS, IFN regulatory factor 7, and IFN stimulated gene 15 expression levels in the muscles of anti\u2010MDA5 DM patients were higher than those of the controls (<jats:italic>P<\/jats:italic>\u00a0&lt;\u00a00.05) but lower than those of antibody\u2010negative DM patients (<jats:italic>P<\/jats:italic>\u00a0&lt;\u00a00.05). The MAVS levels negatively correlated with manual muscle test 8 scores (<jats:italic>r<\/jats:italic>\u00a0=\u00a00.701, <jats:italic>P<\/jats:italic>\u00a0=\u00a00.016).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Compared to antibody\u2010negative DM, we presented a different distribution of the mitochondrial pathology and less severe morphology in anti\u2010MDA5 DM. We also revealed the enhanced but less intensive MAVS\u2010IFN1 signaling pathway activity in muscles of anti\u2010MDA5 DM. Such disparity suggested the potentially different mechanism of muscle injury in two DM groups.<\/jats:p><\/jats:sec>","DOI":"10.1002\/acn3.51311","type":"journal-article","created":{"date-parts":[[2021,2,14]],"date-time":"2021-02-14T13:57:43Z","timestamp":1613311063000},"page":"677-686","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":20,"title":["Mitochondrial morphology and MAVS\u2010IFN1 signaling pathway in muscles of anti\u2010MDA5 dermatomyositis"],"prefix":"10.1002","volume":"8","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5268-9896","authenticated-orcid":false,"given":"Yanyan","family":"Jiang","sequence":"first","affiliation":[{"name":"Department of Neurology Peking University First Hospital Beijing 100034 China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yilin","family":"Liu","sequence":"additional","affiliation":[{"name":"Department of Neurology Peking University First Hospital Beijing 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