{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,19]],"date-time":"2026-03-19T21:07:53Z","timestamp":1773954473845,"version":"3.50.1"},"reference-count":178,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2023,11,22]],"date-time":"2023-11-22T00:00:00Z","timestamp":1700611200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Journal of Neurochemistry"],"published-print":{"date-parts":[[2024,8]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    Polyglutamine spinocerebellar ataxias (PolyQ SCAs) represent a group of monogenetic diseases in which the expanded polyglutamine repeats give rise to a mutated protein. The abnormally expanded polyglutamine protein produces aggregates and toxic species, causing neuronal dysfunction and neuronal death. The main symptoms of these disorders include progressive ataxia, motor dysfunction, oculomotor impairment, and swallowing problems. Nowadays, the current treatments are restricted to symptomatic alleviation, and no existing therapeutic strategies can reduce or stop the disease progression. Even though the origin of these disorders has been associated with polyglutamine\u2010induced toxicity, RNA toxicity has recently gained relevance in polyQ SCAs molecular pathogenesis. Therefore, the research's focus on RNA metabolism has been increasing, especially on RNA\u2010binding proteins (RBPs). The present review summarizes RNA metabolism, exposing the different processes and the main RBPs involved. We also explore the mechanisms by which RBPs are dysregulated in PolyQ SCAs. Finally, possible therapies targeting the RNA metabolism are presented as strategies to reverse neuropathological anomalies and mitigate physical symptoms.\n                    <jats:boxed-text content-type=\"graphic\" position=\"anchor\">\n                      <jats:graphic xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" mimetype=\"image\/png\" position=\"anchor\" specific-use=\"enlarged-web-image\" xlink:href=\"graphic\/jnc16010-fig-0003-m.png\">\n                        <jats:alt-text>image<\/jats:alt-text>\n                      <\/jats:graphic>\n                    <\/jats:boxed-text>\n                  <\/jats:p>","DOI":"10.1111\/jnc.16010","type":"journal-article","created":{"date-parts":[[2023,11,23]],"date-time":"2023-11-23T19:04:51Z","timestamp":1700766291000},"page":"1442-1459","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":10,"title":["From the disruption of\n                    <scp>RNA<\/scp>\n                    metabolism to the targeting of\n                    <scp>RNA<\/scp>\n                    \u2010binding proteins: The case of polyglutamine spinocerebellar ataxias"],"prefix":"10.1111","volume":"168","author":[{"ORCID":"https:\/\/orcid.org\/0009-0004-9783-0058","authenticated-orcid":false,"given":"Tiago","family":"Moreira\u2010Gomes","sequence":"first","affiliation":[{"name":"ABC\u2010RI Algarve Biomedical Center Research Institute  Faro Portugal"},{"name":"Faculdade de Medicina e Ci\u00eancias Biom\u00e9dicas Universidade do Algarve  Faro Portugal"},{"name":"Doctoral Program in Biomedical Sciences, Faculdade de Medicina e Ci\u00eancias Biom\u00e9dicas Universidade do Algarve  Faro Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8312-5292","authenticated-orcid":false,"given":"Cl\u00e9vio","family":"N\u00f3brega","sequence":"additional","affiliation":[{"name":"ABC\u2010RI Algarve Biomedical Center Research Institute  Faro 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Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2023,11,22]]},"reference":[{"key":"e_1_2_11_2_1","doi-asserted-by":"publisher","DOI":"10.1093\/nar\/gkw752"},{"key":"e_1_2_11_3_1","doi-asserted-by":"publisher","DOI":"10.3390\/ijms22084249"},{"key":"e_1_2_11_4_1","doi-asserted-by":"publisher","DOI":"10.1186\/s13024\u2010016\u20100123\u20102"},{"key":"e_1_2_11_5_1","doi-asserted-by":"publisher","DOI":"10.1093\/emboj\/17.5.1497"},{"key":"e_1_2_11_6_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41582\u2010018\u20100051\u20106"},{"key":"e_1_2_11_7_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.274.48.34337"},{"key":"e_1_2_11_8_1","doi-asserted-by":"publisher","DOI":"10.1002\/pdi.606"},{"key":"e_1_2_11_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.neuron.2006.10.028"},{"key":"e_1_2_11_10_1","doi-asserted-by":"publisher","DOI":"10.1093\/nar\/gki1012"},{"key":"e_1_2_11_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.molcel.2004.06.012"},{"key":"e_1_2_11_12_1","doi-asserted-by":"publisher","DOI":"10.1101\/gad.250225.114"},{"key":"e_1_2_11_13_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.1115791"},{"issue":"5","key":"e_1_2_11_14_1","first-page":"2460","article-title":"An 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