{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,16]],"date-time":"2026-10-16T06:21:47Z","timestamp":1792131707618,"version":"4.4.0"},"reference-count":123,"publisher":"Oxford University Press (OUP)","issue":"11","license":[{"start":{"date-parts":[[2023,6,16]],"date-time":"2023-06-16T00:00:00Z","timestamp":1686873600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2023,11,2]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Amyotrophic lateral sclerosis (ALS), the major adult-onset motor neuron disease, has been viewed almost exclusively as a disease of upper and lower motor neurons, with muscle changes interpreted as a consequence of the progressive loss of motor neurons and neuromuscular junctions. This has led to the prevailing view that the involvement of muscle in ALS is only secondary to motor neuron loss. Skeletal muscle and motor neurons reciprocally influence their respective development and constitute a single functional unit. In ALS, multiple studies indicate that skeletal muscle dysfunction might contribute to progressive muscle weakness, as well as to the final demise of neuromuscular junctions and motor neurons. Furthermore, skeletal muscle has been shown to participate in disease pathogenesis of several monogenic diseases closely related to ALS.<\/jats:p>\n                  <jats:p>Here, we move the narrative towards a better appreciation of muscle as a contributor of disease in ALS. We review the various potential roles of skeletal muscle cells in ALS, from passive bystanders to active players in ALS pathophysiology. We also compare ALS to other motor neuron diseases and draw perspectives for future research and treatment.<\/jats:p>","DOI":"10.1093\/brain\/awad202","type":"journal-article","created":{"date-parts":[[2023,6,16]],"date-time":"2023-06-16T14:35:30Z","timestamp":1686926130000},"page":"4425-4436","source":"Crossref","is-referenced-by-count":111,"title":["Skeletal muscle in amyotrophic lateral sclerosis"],"prefix":"10.1093","volume":"146","author":[{"given":"Jeremy M","family":"Shefner","sequence":"first","affiliation":[{"name":"Barrow Neurological Institute , Phoenix, AZ , USA"},{"name":"College of Medicine, University of Arizona, Phoenix , AZ , USA"},{"name":"College of Medicine, Creighton University , Phoenix, AZ , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Antonio","family":"Musaro","sequence":"additional","affiliation":[{"name":"DAHFMO-Unit of Histology and Medical Embryology, Sapienza University of Rome, Laboratory affiliated to Istituto Pasteur Italia\u2014Fondazione Cenci Bolognetti, Scuola Superiore di Studi Avanzati Sapienza (SSAS) , Rome , Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shyuan T","family":"Ngo","sequence":"additional","affiliation":[{"name":"Australian Institute for Bioengineering and Nanotechnology, The University of Queensland , Brisbane , Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christian","family":"Lunetta","sequence":"additional","affiliation":[{"name":"Neurorehabilitation Department, Istituti Clinici Scientifici Maugeri IRCCS , Milan , Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4782-3608","authenticated-orcid":false,"given":"Frederik J","family":"Steyn","sequence":"additional","affiliation":[{"name":"Biomedical Sciences, Faculty of Medicine, The University of Queensland , Brisbane , Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Richard","family":"Robitaille","sequence":"additional","affiliation":[{"name":"D\u00e9partement de neurosciences, CIRCA, Universit\u00e9 de Montr\u00e9al , Montr\u00e9al H7G 1T7 , Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mamede","family":"De Carvalho","sequence":"additional","affiliation":[{"name":"Instituto de Fisiologia, Instituto de Medicina Molecular Jo\u00e3o Lobo Antunes, Faculdade de Medicina, Universidade de Lisboa , Lisbon , Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Seward","family":"Rutkove","sequence":"additional","affiliation":[{"name":"Beth Israel Deaconess Medical Center, Harvard Medical School , Boston, MA , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Albert C","family":"Ludolph","sequence":"additional","affiliation":[{"name":"Department of Neurology, University of Ulm , Ulm , Germany"},{"name":"Deutsches Zentrum f\u00fcr neurodegenerative Erkrankungen (DZNE) , Ulm , Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0902-5824","authenticated-orcid":false,"given":"Luc","family":"Dupuis","sequence":"additional","affiliation":[{"name":"Universit\u00e9 de Strasbourg, Inserm, UMR-S1118, M\u00e9canismes centraux et p\u00e9riph\u00e9riques de la neurod\u00e9g\u00e9n\u00e9rescence, CRBS , Strasbourg , France"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2023,6,16]]},"reference":[{"key":"2023110723334581300_awad202-B1","doi-asserted-by":"crossref","first-page":"465","DOI":"10.1016\/S1474-4422(21)00414-2","article-title":"Emerging insights into the complex genetics and pathophysiology of amyotrophic lateral sclerosis","volume":"21","author":"Goutman","year":"2022","journal-title":"Lancet 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