{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,15]],"date-time":"2026-01-15T21:23:04Z","timestamp":1768512184537,"version":"3.49.0"},"reference-count":68,"publisher":"MDPI AG","issue":"16","license":[{"start":{"date-parts":[[2022,8,11]],"date-time":"2022-08-11T00:00:00Z","timestamp":1660176000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Valencian Agency of Innovation (AVI)","award":["INNTA3\/2021\/26"],"award-info":[{"award-number":["INNTA3\/2021\/26"]}]},{"name":"Valencian Agency of Innovation (AVI)","award":["PI20\/00114"],"award-info":[{"award-number":["PI20\/00114"]}]},{"name":"Valencian Agency of Innovation (AVI)","award":["CIVP19S8119"],"award-info":[{"award-number":["CIVP19S8119"]}]},{"name":"Instituto de Salud Carlos III (ISCIII, Madrid, Spain)","award":["INNTA3\/2021\/26"],"award-info":[{"award-number":["INNTA3\/2021\/26"]}]},{"name":"Instituto de Salud Carlos III (ISCIII, Madrid, Spain)","award":["PI20\/00114"],"award-info":[{"award-number":["PI20\/00114"]}]},{"name":"Instituto de Salud Carlos III (ISCIII, Madrid, Spain)","award":["CIVP19S8119"],"award-info":[{"award-number":["CIVP19S8119"]}]},{"name":"European Development Regional Fund","award":["INNTA3\/2021\/26"],"award-info":[{"award-number":["INNTA3\/2021\/26"]}]},{"name":"European Development Regional Fund","award":["PI20\/00114"],"award-info":[{"award-number":["PI20\/00114"]}]},{"name":"European Development Regional Fund","award":["CIVP19S8119"],"award-info":[{"award-number":["CIVP19S8119"]}]},{"name":"Fundaci\u00f3n Ram\u00f3n Areces","award":["INNTA3\/2021\/26"],"award-info":[{"award-number":["INNTA3\/2021\/26"]}]},{"name":"Fundaci\u00f3n Ram\u00f3n Areces","award":["PI20\/00114"],"award-info":[{"award-number":["PI20\/00114"]}]},{"name":"Fundaci\u00f3n Ram\u00f3n Areces","award":["CIVP19S8119"],"award-info":[{"award-number":["CIVP19S8119"]}]},{"name":"Asociaci\u00f3n Valenciana de Enfermedad de Huntington (AVAEH)","award":["INNTA3\/2021\/26"],"award-info":[{"award-number":["INNTA3\/2021\/26"]}]},{"name":"Asociaci\u00f3n Valenciana de Enfermedad de Huntington (AVAEH)","award":["PI20\/00114"],"award-info":[{"award-number":["PI20\/00114"]}]},{"name":"Asociaci\u00f3n Valenciana de Enfermedad de Huntington (AVAEH)","award":["CIVP19S8119"],"award-info":[{"award-number":["CIVP19S8119"]}]},{"name":"European Development Regional Fund (FEDER)","award":["INNTA3\/2021\/26"],"award-info":[{"award-number":["INNTA3\/2021\/26"]}]},{"name":"European Development Regional Fund (FEDER)","award":["PI20\/00114"],"award-info":[{"award-number":["PI20\/00114"]}]},{"name":"European Development Regional Fund (FEDER)","award":["CIVP19S8119"],"award-info":[{"award-number":["CIVP19S8119"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Huntington\u2019s disease (HD) is an autosomal dominant neurodegenerative disorder, of the so-called minority diseases, due to its low prevalence. It is caused by an abnormally long track of glutamines (polyQs) in mutant huntingtin (mHtt), which makes the protein toxic and prone to aggregation. Many pathways of clearance of badly-folded proteins are disrupted in neurons of patients with HD. In this work, we show that one Mn(II) quinone complex (4QMn), designed to work as an artificial superoxide dismutase, is able to activate both the ubiquitin-proteasome system and the autophagy pathway in vitro and in vivo models of HD. Activation of these pathways degrades mHtt and other protein-containing polyQs, which restores proteostasis in these models. Hence, we propose 4QMn as a potential drug to develop a therapy to treat HD.<\/jats:p>","DOI":"10.3390\/ijms23168936","type":"journal-article","created":{"date-parts":[[2022,8,11]],"date-time":"2022-08-11T05:43:57Z","timestamp":1660196637000},"page":"8936","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["Mn(II) Quinoline Complex (4QMn) Restores Proteostasis and Reduces Toxicity in Experimental Models of Huntington\u2019s Disease"],"prefix":"10.3390","volume":"23","author":[{"given":"Mari\u00e1n","family":"Merino","sequence":"first","affiliation":[{"name":"Bionos Biotech SL, Biopolo Hospital La Fe, 46026 Valencia, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mar\u00eda Dolores","family":"Sequedo","sequence":"additional","affiliation":[{"name":"Laboratory of Molecular, Cellular and Genomic Biomedicine, Instituto de Investigaci\u00f3n Sanitaria La Fe, 46026 Valencia, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ana Virginia","family":"S\u00e1nchez-S\u00e1nchez","sequence":"additional","affiliation":[{"name":"Bionos Biotech SL, Biopolo Hospital La Fe, 46026 Valencia, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M\u00aa Paz","family":"Clares","sequence":"additional","affiliation":[{"name":"Departamento de Qu\u00edmica Org\u00e1nica e Inorg\u00e1nica, Instituto de Ciencia Molecular, Universidad de Valencia, 46980 Valencia, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Enrique","family":"Garc\u00eda-Espa\u00f1a","sequence":"additional","affiliation":[{"name":"Departamento de Qu\u00edmica Org\u00e1nica e Inorg\u00e1nica, Instituto de Ciencia Molecular, Universidad de Valencia, 46980 Valencia, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1594-0033","authenticated-orcid":false,"given":"Rafael P.","family":"V\u00e1zquez-Manrique","sequence":"additional","affiliation":[{"name":"Laboratory of Molecular, Cellular and Genomic Biomedicine, Instituto de Investigaci\u00f3n Sanitaria La Fe, 46026 Valencia, Spain"},{"name":"Centro de Investigaci\u00f3n Biom\u00e9dica en Red de Enfermedades Raras (CIBERER), 28029 Madrid, Spain"},{"name":"Joint Unit for Rare Diseases IIS La Fe-CIPF, 46012 Valencia, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jos\u00e9 L.","family":"Mullor","sequence":"additional","affiliation":[{"name":"Bionos Biotech SL, Biopolo Hospital La Fe, 46026 Valencia, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2022,8,11]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"1279","DOI":"10.1038\/nsmb.1700","article-title":"The chaperonin TRiC blocks a huntingtin sequence element that promotes the conformational switch to aggregation","volume":"16","author":"Tam","year":"2009","journal-title":"Nat. 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