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Alliance"],"published-print":{"date-parts":[[2023,3]]},"abstract":"<jats:p>\n                    Spinal muscular atrophy (SMA) is a congenital neuromuscular disease caused by the mutation or deletion of the\n                    <jats:italic>survival motor neuron 1 (SMN1)<\/jats:italic>\n                    gene. Although the primary cause of progressive muscle atrophy in SMA has classically been considered the degeneration of motor neurons, recent studies have indicated a skeletal muscle\u2013specific pathological phenotype such as impaired mitochondrial function and enhanced cell death. Here, we found that the down-regulation of SMN causes mitochondrial dysfunction and subsequent cell death in in vitro models of skeletal myogenesis with both a murine C2C12 cell line and human induced pluripotent stem cells. During myogenesis, SMN binds to the upstream genomic regions of MYOD1 and microRNA (miR)-1 and miR-206. Accordingly, the loss of SMN down-regulates these miRs, whereas supplementation of the miRs recovers the mitochondrial function, cell survival, and myotube formation of SMN-deficient C2C12, indicating the SMN-miR axis is essential for myogenic metabolic maturation. In addition, the introduction of the miRs into ex vivo muscle stem cells derived from \u03947-SMA mice caused myotube formation and muscle contraction. In conclusion, our data revealed novel transcriptional roles of SMN during myogenesis, providing an alternative muscle-oriented therapeutic strategy for SMA patients.\n                  <\/jats:p>","DOI":"10.26508\/lsa.202201457","type":"journal-article","created":{"date-parts":[[2023,1,5]],"date-time":"2023-01-05T10:45:13Z","timestamp":1672915513000},"page":"e202201457","update-policy":"https:\/\/doi.org\/10.26508\/lsa.crossmarkpolicy","source":"Crossref","is-referenced-by-count":25,"title":["SMN promotes mitochondrial metabolic maturation during myogenesis by regulating the MYOD-miRNA axis"],"prefix":"10.26508","volume":"6","author":[{"given":"Akihiro","family":"Ikenaka","sequence":"first","affiliation":[{"name":"Department of Clinical Application, Center for iPS Cell Research and Application, Kyoto University, Kyoto, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yohko","family":"Kitagawa","sequence":"additional","affiliation":[{"name":"Department of Clinical 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