{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T04:52:12Z","timestamp":1787028732743,"version":"build-2736575974"},"reference-count":309,"publisher":"MDPI AG","issue":"7","license":[{"start":{"date-parts":[[2021,7,16]],"date-time":"2021-07-16T00:00:00Z","timestamp":1626393600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100004587","name":"Instituto de Salud Carlos III","doi-asserted-by":"publisher","award":["PI19\/00175"],"award-info":[{"award-number":["PI19\/00175"]}],"id":[{"id":"10.13039\/501100004587","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100019124","name":"Diputaci\u00f3n Foral de Gipuzkoa","doi-asserted-by":"publisher","award":["2020-CIEN-000057-01"],"award-info":[{"award-number":["2020-CIEN-000057-01"]}],"id":[{"id":"10.13039\/501100019124","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100010585","name":"Osasun Saila, Eusko Jaurlaritzako","doi-asserted-by":"publisher","award":["2020111032"],"award-info":[{"award-number":["2020111032"]}],"id":[{"id":"10.13039\/501100010585","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JPM"],"abstract":"<jats:p>Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder that leads to progressive degeneration of motor neurons (MNs) and severe muscle atrophy without effective treatment. Most research on ALS has been focused on the study of MNs and supporting cells of the central nervous system. Strikingly, the recent observations of pathological changes in muscle occurring before disease onset and independent from MN degeneration have bolstered the interest for the study of muscle tissue as a potential target for delivery of therapies for ALS. Skeletal muscle has just been described as a tissue with an important secretory function that is toxic to MNs in the context of ALS. Moreover, a fine-tuning balance between biosynthetic and atrophic pathways is necessary to induce myogenesis for muscle tissue repair. Compromising this response due to primary metabolic abnormalities in the muscle could trigger defective muscle regeneration and neuromuscular junction restoration, with deleterious consequences for MNs and thereby hastening the development of ALS. However, it remains puzzling how backward signaling from the muscle could impinge on MN death. This review provides a comprehensive analysis on the current state-of-the-art of the role of the skeletal muscle in ALS, highlighting its contribution to the neurodegeneration in ALS through backward-signaling processes as a newly uncovered mechanism for a peripheral etiopathogenesis of the disease.<\/jats:p>","DOI":"10.3390\/jpm11070671","type":"journal-article","created":{"date-parts":[[2021,7,18]],"date-time":"2021-07-18T21:16:48Z","timestamp":1626643008000},"page":"671","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":46,"title":["The Skeletal Muscle Emerges as a New Disease Target in Amyotrophic Lateral Sclerosis"],"prefix":"10.3390","volume":"11","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4221-8289","authenticated-orcid":false,"given":"Oihane","family":"Pikatza-Menoio","sequence":"first","affiliation":[{"name":"Neuromuscular Diseases Group, Neurosciences Area, Biodonostia Health Research Institute, 20014 Donostia\/San Sebasti\u00e1n, Spain"},{"name":"CIBERNED, Carlos III Institute, Spanish Ministry of Economy & Competitiveness, 28031 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4782-6005","authenticated-orcid":false,"given":"Amaia","family":"Elicegui","sequence":"additional","affiliation":[{"name":"Neuromuscular Diseases Group, Neurosciences Area, Biodonostia Health Research Institute, 20014 Donostia\/San Sebasti\u00e1n, Spain"},{"name":"CIBERNED, Carlos III Institute, Spanish Ministry of Economy & Competitiveness, 28031 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8651-6320","authenticated-orcid":false,"given":"Xabier","family":"Bengoetxea","sequence":"additional","affiliation":[{"name":"Neuromuscular Diseases Group, Neurosciences Area, Biodonostia Health Research Institute, 20014 Donostia\/San Sebasti\u00e1n, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Neia","family":"Naldaiz-Gastesi","sequence":"additional","affiliation":[{"name":"Neuromuscular Diseases Group, Neurosciences Area, Biodonostia Health Research Institute, 20014 Donostia\/San Sebasti\u00e1n, Spain"},{"name":"CIBERNED, Carlos III Institute, Spanish Ministry of Economy & Competitiveness, 28031 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9509-4032","authenticated-orcid":false,"given":"Adolfo","family":"L\u00f3pez de Munain","sequence":"additional","affiliation":[{"name":"Neuromuscular Diseases Group, Neurosciences Area, Biodonostia Health Research Institute, 20014 Donostia\/San Sebasti\u00e1n, Spain"},{"name":"CIBERNED, Carlos III Institute, Spanish Ministry of Economy & Competitiveness, 28031 Madrid, Spain"},{"name":"Department of Neurology, Donostialdea Integrated Health Organization, Osakidetza Basque Health Service, 20014 Donostia\/San Sebasti\u00e1n, Spain"},{"name":"Department of Neurosciences, Faculty of Medicine and Nursery, University of the Basque Country UPV-EHU, 20014 Donostia\/San Sebasti\u00e1n, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gorka","family":"Gerenu","sequence":"additional","affiliation":[{"name":"Neuromuscular Diseases Group, Neurosciences Area, Biodonostia Health Research Institute, 20014 Donostia\/San Sebasti\u00e1n, Spain"},{"name":"CIBERNED, Carlos III Institute, Spanish Ministry of Economy & Competitiveness, 28031 Madrid, Spain"},{"name":"Department of Physiology, University of the Basque Country UPV-EHU, 48940 Leioa, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francisco Javier","family":"Gil-Bea","sequence":"additional","affiliation":[{"name":"Neuromuscular Diseases Group, Neurosciences Area, Biodonostia Health Research Institute, 20014 Donostia\/San Sebasti\u00e1n, Spain"},{"name":"CIBERNED, Carlos III Institute, Spanish Ministry of Economy & Competitiveness, 28031 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3254-0365","authenticated-orcid":false,"given":"Sonia","family":"Alonso-Mart\u00edn","sequence":"additional","affiliation":[{"name":"Neuromuscular Diseases Group, Neurosciences Area, Biodonostia Health Research Institute, 20014 Donostia\/San Sebasti\u00e1n, Spain"},{"name":"CIBERNED, Carlos III Institute, Spanish Ministry of Economy & Competitiveness, 28031 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2021,7,16]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"409","DOI":"10.1055\/s-0036-1586263","article-title":"Neuromuscular Diseases","volume":"36","author":"Morrison","year":"2016","journal-title":"Semin. 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