{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,29]],"date-time":"2026-09-29T07:06:03Z","timestamp":1790665563862,"version":"4.1.0"},"reference-count":53,"publisher":"Oxford University Press (OUP)","issue":"1","license":[{"start":{"date-parts":[[2024,11,6]],"date-time":"2024-11-06T00:00:00Z","timestamp":1730851200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"DOI":"10.13039\/100005202","name":"Muscular Dystrophy Association","doi-asserted-by":"publisher","award":["963652"],"award-info":[{"award-number":["963652"]}],"id":[{"id":"10.13039\/100005202","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000024","name":"Canadian Institutes of Health Research","doi-asserted-by":"publisher","award":["PJT-186300"],"award-info":[{"award-number":["PJT-186300"]}],"id":[{"id":"10.13039\/501100000024","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100007721","name":"Cure SMA","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100007721","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000223","name":"Muscular Dystrophy Canada","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100000223","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2025,1,23]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Spinal muscular atrophy (SMA) is characterized by low levels of the ubiquitously expressed Survival Motor Neuron (SMN) protein, leading to progressive muscle weakness and atrophy. Skeletal muscle satellite cells play a crucial role in muscle fiber maintenance, repair, and remodelling. While the effects of SMN depletion in muscle are well documented, its precise role in satellite cell function remains largely unclear. Using the Smn2B\/\u2212 mouse model, we investigated SMN-depleted satellite cell biology through single fiber culture studies. Myofibers from Smn2B\/\u2212 mice were smaller in size, shorter in length, had reduced myonuclear domain size, and reduced sub-synaptic myonuclear clusters\u2014all suggesting impaired muscle function and integrity. These changes were accompanied by a reduction in the number of myonuclei in myofibers from Smn2B\/\u2212 mice across all disease stages examined. Although the number of satellite cells in myofibers was significantly reduced, those remaining retained their capacity for myogenic activation and proliferation. These findings support the idea that a dysregulated myogenic process could be occurring as early in muscle stem cells during muscle formation and maturation in SMA. Targeting those pathways could offer additional options for combinatorial therapies for SMA.<\/jats:p>","DOI":"10.1093\/hmg\/ddae162","type":"journal-article","created":{"date-parts":[[2024,11,1]],"date-time":"2024-11-01T16:20:06Z","timestamp":1730478006000},"page":"21-31","source":"Crossref","is-referenced-by-count":7,"title":["SMN depletion impairs skeletal muscle formation and maturation in a mouse model of SMA"],"prefix":"10.1093","volume":"34","author":[{"given":"Hong","family":"Liu","sequence":"first","affiliation":[{"name":"Regenerative Medicine Program, Ottawa Hospital Research Institute , 501 Smyth Road, Ottawa, ON K1H 8L6,","place":["Canada"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lucia","family":"Chehade","sequence":"additional","affiliation":[{"name":"Regenerative Medicine Program, Ottawa Hospital Research Institute , 501 Smyth Road, Ottawa, ON K1H 8L6,","place":["Canada"]},{"name":"Department of Cellular and Molecular Medicine, University of Ottawa , 451 Smyth Road, Ottawa, ON K1H 8M5,","place":["Canada"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marc-Olivier","family":"Deguise","sequence":"additional","affiliation":[{"name":"Regenerative Medicine Program, Ottawa Hospital Research Institute , 501 Smyth Road, Ottawa, ON K1H 8L6,","place":["Canada"]},{"name":"Center for Neuromuscular Disease, University of Ottawa , 451 Smyth Road, Ottawa, ON K1H 8M5,","place":["Canada"]},{"name":"Department of Pediatrics, Children\u2019s Hospital of Eastern Ontario , 401 Smyth Road, Ottawa, ON K1H 8L1,","place":["Canada"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yves","family":"De Repentigny","sequence":"additional","affiliation":[{"name":"Regenerative Medicine Program, Ottawa Hospital Research Institute , 501 Smyth Road, Ottawa, ON K1H 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