{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,20]],"date-time":"2026-08-20T00:58:11Z","timestamp":1787187491426,"version":"build-2736575974"},"reference-count":42,"publisher":"Public Library of Science (PLoS)","issue":"8","license":[{"start":{"date-parts":[[2024,8,1]],"date-time":"2024-08-01T00:00:00Z","timestamp":1722470400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"National Center of Neurology and Psychiatry of Japan","award":["29-4 and 2-5"],"award-info":[{"award-number":["29-4 and 2-5"]}]},{"name":"Practical Research Project for Rare\/Diseases","award":["20dk0310086"],"award-info":[{"award-number":["20dk0310086"]}]},{"DOI":"10.13039\/100009619","name":"Japan Agency for Medical Research and Development","doi-asserted-by":"crossref","award":["JPMJMS 23zf0127001h0003"],"award-info":[{"award-number":["JPMJMS 23zf0127001h0003"]}],"id":[{"id":"10.13039\/100009619","id-type":"DOI","asserted-by":"crossref"}]},{"name":"Ministry of Health, Labor and Welfare of Japan","award":["H29-nanchitou(nan)-ippan-030 and 20CF1036"],"award-info":[{"award-number":["H29-nanchitou(nan)-ippan-030 and 20CF1036"]}]},{"name":"Grant-in-Aid for Challenging Exploratory Research","award":["20K21563"],"award-info":[{"award-number":["20K21563"]}]},{"DOI":"10.13039\/501100001700","name":"Japanese Ministry of Education, Culture, Sports, Science and Technology","doi-asserted-by":"crossref","award":["18K07519"],"award-info":[{"award-number":["18K07519"]}],"id":[{"id":"10.13039\/501100001700","id-type":"DOI","asserted-by":"crossref"}]},{"name":"Cooperative Research Project Program of the Joint Usage\/Research Center at the Institute of Development, Aging and Cancer, Tohoku University"}],"content-domain":{"domain":["www.plosone.org"],"crossmark-restriction":false},"short-container-title":["PLoS ONE"],"abstract":"<jats:p>Sporadic inclusion body myositis (sIBM) is a muscle disease in older people and is characterized by inflammatory cell invasion into intact muscle fibers and rimmed vacuoles. The pathomechanism of sIBM is not fully elucidated yet, and controversy exists as to whether sIBM is a primary autoimmune disease or a degenerative muscle disease with secondary inflammation. Previously, we established a method of collecting CD56-positive myoblasts from human skeletal muscle biopsy samples. We hypothesized that the myoblasts derived from these patients are useful to see the cell-autonomous pathomechanism of sIBM. With these resources, myoblasts were differentiated into myotubes, and the expression profiles of cell-autonomous pathology of sIBM were analyzed. Myoblasts from three sIBM cases and six controls were differentiated into myotubes. In the RNA-sequencing analysis of these \u201cmyotube\u201d samples, 104 differentially expressed genes (DEGs) were found to be significantly upregulated by more than twofold in sIBM, and 13 DEGs were downregulated by less than twofold. For muscle biopsy samples, a comparative analysis was conducted to determine the extent to which \u201cbiopsy\u201d and \u201cmyotube\u201d samples differed. Fifty-three DEGs were extracted of which 32 (60%) had opposite directions of expression change (e.g., increased in biopsy vs decreased in myotube). Apolipoprotein E (apoE) and transmembrane protein 8C (TMEM8C or MYMK) were commonly upregulated in muscle biopsies and myotubes from sIBM. ApoE and myogenin protein levels were upregulated in sIBM. Given that enrichment analysis also captured changes in muscle contraction and development, the triggering of muscle atrophy signaling and abnormal muscle differentiation via MYMK or myogenin may be involved in the pathogenesis of sIBM. The presence of DEGs in sIBM suggests that the myotubes formed from sIBM-derived myoblasts revealed the existence of muscle cell-autonomous degeneration in sIBM. The catalog of DEGs will be an important resource for future studies on the pathogenesis of sIBM focusing on primary muscle degeneration.<\/jats:p>","DOI":"10.1371\/journal.pone.0306021","type":"journal-article","created":{"date-parts":[[2024,8,1]],"date-time":"2024-08-01T17:36:24Z","timestamp":1722533784000},"page":"e0306021","update-policy":"https:\/\/doi.org\/10.1371\/journal.pone.corrections_policy","source":"Crossref","is-referenced-by-count":2,"title":["Sporadic inclusion body myositis-derived myotube culture revealed muscle cell-autonomous expression 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