{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,29]],"date-time":"2026-07-29T22:18:11Z","timestamp":1785363491629,"version":"3.55.0"},"reference-count":164,"publisher":"MDPI AG","issue":"17","license":[{"start":{"date-parts":[[2020,9,3]],"date-time":"2020-09-03T00:00:00Z","timestamp":1599091200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100004200","name":"Stiftelsen Olle Engkvist Byggm\u00e4stare","doi-asserted-by":"publisher","award":["188-0100"],"award-info":[{"award-number":["188-0100"]}],"id":[{"id":"10.13039\/501100004200","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100010198","name":"Ministerio de Econom\u00eda, Industria y Competitividad, Gobierno de Espa\u00f1a","doi-asserted-by":"publisher","award":["RTI2018-098645-B-100"],"award-info":[{"award-number":["RTI2018-098645-B-100"]}],"id":[{"id":"10.13039\/501100010198","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Neuromuscular disorders (NMDs) affect 1 in 3000 people worldwide. There are more than 150 different types of NMDs, where the common feature is the loss of muscle strength. These disorders are classified according to their neuroanatomical location, as motor neuron diseases, peripheral nerve diseases, neuromuscular junction diseases, and muscle diseases. Over the years, numerous studies have pointed to protein homeostasis as a crucial factor in the development of these fatal diseases. The ubiquitin\u2013proteasome system (UPS) plays a fundamental role in maintaining protein homeostasis, being involved in protein degradation, among other cellular functions. Through a cascade of enzymatic reactions, proteins are ubiquitinated, tagged, and translocated to the proteasome to be degraded. Within the ubiquitin system, we can find three main groups of enzymes: E1 (ubiquitin-activating enzymes), E2 (ubiquitin-conjugating enzymes), and E3 (ubiquitin\u2013protein ligases). Only the ubiquitinated proteins with specific chain linkages (such as K48) will be degraded by the UPS. In this review, we describe the relevance of this system in NMDs, summarizing the UPS proteins that have been involved in pathological conditions and neuromuscular disorders, such as Spinal Muscular Atrophy (SMA), Charcot\u2013Marie\u2013Tooth disease (CMT), or Duchenne Muscular Dystrophy (DMD), among others. A better knowledge of the processes involved in the maintenance of proteostasis may pave the way for future progress in neuromuscular disorder studies and treatments.<\/jats:p>","DOI":"10.3390\/ijms21176429","type":"journal-article","created":{"date-parts":[[2020,9,3]],"date-time":"2020-09-03T11:22:43Z","timestamp":1599132163000},"page":"6429","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":27,"title":["The Ubiquitin Proteasome System in Neuromuscular Disorders: Moving Beyond Movement"],"prefix":"10.3390","volume":"21","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9000-3787","authenticated-orcid":false,"given":"Sara","family":"Bachiller","sequence":"first","affiliation":[{"name":"Experimental Neuroinflammation Laboratory, Department of Experimental Medical Science, Lund University, S\u00f6lvegatan 19, 221 84 Lund, Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7475-8653","authenticated-orcid":false,"given":"Isabel M.","family":"Alonso-Bellido","sequence":"additional","affiliation":[{"name":"Departamento de Bioqu\u00edmica y Biolog\u00eda Molecular, Facultad de Farmacia, Universidad de Sevilla\/Instituto de Biomedicina de Sevilla-Hospital Universitario Virgen del Roc\u00edo\/CSIC\/Universidad de Sevilla, 41012 Sevilla, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4932-7429","authenticated-orcid":false,"given":"Luis Miguel","family":"Real","sequence":"additional","affiliation":[{"name":"Unidad Cl\u00ednica de Enfermedades Infecciosas, Hospital Universitario de Valme, 41014 Sevilla, Spain"},{"name":"Departamento de Especialidades Quir\u00fargicas, Bioqu\u00edmica e Inmunolog\u00eda, Facultad de Medicina, 29071 Universidad de M\u00e1laga, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2427-0748","authenticated-orcid":false,"given":"Eva Mar\u00eda","family":"P\u00e9rez-Villegas","sequence":"additional","affiliation":[{"name":"Departamento de Fisiolog\u00eda, Anatom\u00eda y Biolog\u00eda Celular, Universidad Pablo de Olavide, 41013 Sevilla, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1137-8706","authenticated-orcid":false,"given":"Jos\u00e9 Luis","family":"Venero","sequence":"additional","affiliation":[{"name":"Departamento de Bioqu\u00edmica y Biolog\u00eda Molecular, Facultad de Farmacia, Universidad de Sevilla\/Instituto de Biomedicina de Sevilla-Hospital Universitario Virgen del Roc\u00edo\/CSIC\/Universidad de Sevilla, 41012 Sevilla, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3651-3444","authenticated-orcid":false,"given":"Tomas","family":"Deierborg","sequence":"additional","affiliation":[{"name":"Experimental Neuroinflammation Laboratory, Department of Experimental Medical Science, Lund University, S\u00f6lvegatan 19, 221 84 Lund, Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9452-7828","authenticated-orcid":false,"given":"Jos\u00e9 \u00c1ngel","family":"Armengol","sequence":"additional","affiliation":[{"name":"Departamento de Fisiolog\u00eda, Anatom\u00eda y Biolog\u00eda Celular, Universidad Pablo de Olavide, 41013 Sevilla, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5142-9972","authenticated-orcid":false,"given":"Roc\u00edo","family":"Ruiz","sequence":"additional","affiliation":[{"name":"Departamento de Bioqu\u00edmica y Biolog\u00eda Molecular, Facultad de Farmacia, Universidad de Sevilla\/Instituto de Biomedicina de Sevilla-Hospital Universitario Virgen del Roc\u00edo\/CSIC\/Universidad de Sevilla, 41012 Sevilla, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2020,9,3]]},"reference":[{"key":"ref_1","first-page":"35","article-title":"Report by the Spanish Foundation for the Brain on the social impact of amyotrophic lateral sclerosis and other neuromuscular disorders","volume":"33","author":"Camacho","year":"2018","journal-title":"Neurol. 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