{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,12]],"date-time":"2025-10-12T02:23:10Z","timestamp":1760235790883,"version":"build-2065373602"},"reference-count":44,"publisher":"MDPI AG","issue":"10","license":[{"start":{"date-parts":[[2021,9,24]],"date-time":"2021-09-24T00:00:00Z","timestamp":1632441600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100002426","name":"Fondazione Telethon","doi-asserted-by":"publisher","award":["GGP20101"],"award-info":[{"award-number":["GGP20101"]}],"id":[{"id":"10.13039\/501100002426","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Brain Sciences"],"abstract":"<jats:p>Huntington\u2019s disease (HD) is a rare hereditary neurodegenerative disorder characterized by multiple metabolic dysfunctions including defects in mitochondrial homeostasis and functions. Although we have recently reported age-related changes in the respiratory capacities in different brain areas in HD mice, the precise mechanisms of how mitochondria become compromised in HD are still poorly understood. In this study, we investigated mRNA and protein levels of selected subunits of electron transport system (ETS) complexes and ATP-synthase in the cortex and striatum of symptomatic R6\/2 mice. Our findings reveal a brain-region-specific differential expression of both nuclear and mitochondrial-encoded ETS components, indicating defects of transcription, translation and\/or mitochondrial import of mitochondrial ETS components in R6\/2 mouse brains.<\/jats:p>","DOI":"10.3390\/brainsci11101267","type":"journal-article","created":{"date-parts":[[2021,9,24]],"date-time":"2021-09-24T20:15:32Z","timestamp":1632514532000},"page":"1267","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Brain Region and Cell Compartment Dependent Regulation of Electron Transport System Components in Huntington\u2019s Disease Model Mice"],"prefix":"10.3390","volume":"11","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2889-0151","authenticated-orcid":false,"given":"Johannes","family":"Burtscher","sequence":"first","affiliation":[{"name":"Institute of Sport Sciences, University of Lausanne, CH-1015 Lausanne, Switzerland"},{"name":"Department of Biomedical Sciences, University of Lausanne, CH-1015 Lausanne, Switzerland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Giuseppe","family":"Pepe","sequence":"additional","affiliation":[{"name":"IRCCS Neuromed, 86077 Pozzilli, IS, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Federico","family":"Marracino","sequence":"additional","affiliation":[{"name":"IRCCS Neuromed, 86077 Pozzilli, IS, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Luca","family":"Capocci","sequence":"additional","affiliation":[{"name":"IRCCS Neuromed, 86077 Pozzilli, IS, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Susy","family":"Giova","sequence":"additional","affiliation":[{"name":"IRCCS Neuromed, 86077 Pozzilli, IS, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8081-4423","authenticated-orcid":false,"given":"Gr\u00e9goire P.","family":"Millet","sequence":"additional","affiliation":[{"name":"Institute of Sport Sciences, University of Lausanne, CH-1015 Lausanne, Switzerland"},{"name":"Department of Biomedical Sciences, University of Lausanne, CH-1015 Lausanne, Switzerland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alba","family":"Di Pardo","sequence":"additional","affiliation":[{"name":"IRCCS Neuromed, 86077 Pozzilli, IS, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Vittorio","family":"Maglione","sequence":"additional","affiliation":[{"name":"IRCCS Neuromed, 86077 Pozzilli, IS, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2021,9,24]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"218","DOI":"10.1016\/S0140-6736(07)60111-1","article-title":"Huntington\u2019s disease","volume":"369","author":"Walker","year":"2007","journal-title":"Lancet"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"127","DOI":"10.3389\/fphys.2017.00127","article-title":"Transcriptional Signature of an Altered Purine Metabolism in the Skeletal Muscle of a Huntington\u2019s Disease Mouse Model","volume":"8","author":"Mielcarek","year":"2017","journal-title":"Front. 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