{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,24]],"date-time":"2026-10-24T01:33:08Z","timestamp":1792805588832,"version":"4.5.0"},"reference-count":138,"publisher":"MDPI AG","issue":"4","license":[{"start":{"date-parts":[[2025,4,1]],"date-time":"2025-04-01T00:00:00Z","timestamp":1743465600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Ministero dell'universit\u00e0 e della ricerca","award":["ECS00000041"],"award-info":[{"award-number":["ECS00000041"]}],"id":[{"id":"https:\/\/ror.org\/0341vw408","id-type":"ROR","asserted-by":"crossref"}]},{"name":"Ministero dell'universit\u00e0 e della ricerca","award":["E19J21012890001"],"award-info":[{"award-number":["E19J21012890001"]}],"id":[{"id":"https:\/\/ror.org\/0341vw408","id-type":"ROR","asserted-by":"crossref"}]},{"name":"Domp\u00e9 (Italy)","award":["B19J23000180005"],"award-info":[{"award-number":["B19J23000180005"]}],"id":[{"id":"https:\/\/ror.org\/04erezm33","id-type":"ROR","asserted-by":"crossref"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Life"],"abstract":"<jats:p>Huntington\u2019s disease (HD) is a detrimental neurodegenerative disease caused by the expansion of a CAG triplet in the HTT gene. This mutation leads to the production of mutant Huntingtin (Htt) protein with toxic gain-of-function. The mHtt is responsible in several ways for the establishment of an intricate pathogenetic scenario in affected cells, particularly in HD neurons. Among the features of HD, oxidative stress plays a relevant role in the progression of the disease at the cellular level. Mitochondrial dysfunction, bioenergetic deficits, Reactive Oxygen Species (ROS) production, neuroinflammation, and general reduction of antioxidant levels are all involved in the promotion of a toxic oxidative environment, eventually causing cell death. Nonetheless, neuronal cells exert antioxidant molecules to build up defense mechanisms. Key components of these defensive mechanisms are the nuclear factor erythroid 2-related factor 2 (NRF2) and peroxisome proliferator-activated receptor gamma coactivator-1 \u03b1 (PGC-1\u03b1). Thus, this review aims to describe the involvement of oxidative stress in HD by exploring the roles of NRF2 and PGC-1\u03b1, crucial actors in this play. Finally, antioxidant therapeutic strategies targeting such markers are discussed.<\/jats:p>","DOI":"10.3390\/life15040577","type":"journal-article","created":{"date-parts":[[2025,4,2]],"date-time":"2025-04-02T07:47:44Z","timestamp":1743580064000},"page":"577","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":18,"title":["Antioxidant and Anti-Inflammatory Defenses in Huntington\u2019s Disease: Roles of NRF2 and PGC-1\u03b1, and Therapeutic Strategies"],"prefix":"10.3390","volume":"15","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9695-6274","authenticated-orcid":false,"given":"Francesco","family":"D\u2019Egidio","sequence":"first","affiliation":[{"name":"Department of Life, Health and Environmental Sciences, University of L\u2019Aquila, 67100 L\u2019Aquila, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elvira","family":"Qosja","sequence":"additional","affiliation":[{"name":"Department of Clinical Disciplines, University \u2018Alexander Xhuvani\u2019 of Elbasan, 3001 Elbasan, Albania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0005-5416-4187","authenticated-orcid":false,"given":"Fabrizio","family":"Ammannito","sequence":"additional","affiliation":[{"name":"Department of Life, Health and Environmental Sciences, University of L\u2019Aquila, 67100 L\u2019Aquila, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6661-8116","authenticated-orcid":false,"given":"Skender","family":"Topi","sequence":"additional","affiliation":[{"name":"Department of Clinical Disciplines, University \u2018Alexander Xhuvani\u2019 of Elbasan, 3001 Elbasan, Albania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3693-840X","authenticated-orcid":false,"given":"Michele","family":"d\u2019Angelo","sequence":"additional","affiliation":[{"name":"Department of Life, Health and Environmental Sciences, University of L\u2019Aquila, 67100 L\u2019Aquila, Italy"},{"name":"Sbarro Institute for Cancer Research and Molecular Medicine, Temple University, Philadelphia, PA 19122, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2737-7970","authenticated-orcid":false,"given":"Annamaria","family":"Cimini","sequence":"additional","affiliation":[{"name":"Department of Life, Health and Environmental Sciences, University of L\u2019Aquila, 67100 L\u2019Aquila, Italy"},{"name":"Sbarro Institute for Cancer Research and Molecular Medicine, Temple University, Philadelphia, PA 19122, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7005-3218","authenticated-orcid":false,"given":"Vanessa","family":"Castelli","sequence":"additional","affiliation":[{"name":"Department of Life, Health and Environmental Sciences, University of L\u2019Aquila, 67100 L\u2019Aquila, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2025,4,1]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"83","DOI":"10.1016\/S1474-4422(10)70245-3","article-title":"Huntington\u2019s Disease: From Molecular Pathogenesis to Clinical Treatment","volume":"10","author":"Ross","year":"2011","journal-title":"Lancet Neurol."},{"key":"ref_2","doi-asserted-by":"crossref","unstructured":"D\u2019Egidio, F., Castelli, V., Cimini, A., and d\u2019Angelo, M. 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