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Among them, reduced cholesterol biosynthesis has been detected in the HD mouse brain from pre\u2010symptomatic stages, leading to diminished cholesterol synthesis, particularly in the striatum. In addition, systemic injection of cholesterol\u2010loaded brain\u2010permeable nanoparticles ameliorates synaptic and cognitive function in a transgenic mouse model of HD. To identify an appropriate treatment regimen and gain mechanistic insights into the beneficial activity of exogenous cholesterol in the HD brain, we employed osmotic mini\u2010pumps to infuse three escalating doses of cholesterol directly into the striatum of HD mice in a continuous and rate\u2010controlled manner. All tested doses prevented cognitive decline, while amelioration of disease\u2010related motor defects was dose\u2010dependent. In parallel, we found morphological and functional recovery of synaptic transmission involving both excitatory and inhibitory synapses of striatal medium spiny neurons. The treatment also enhanced endogenous cholesterol biosynthesis and clearance of mutant Huntingtin aggregates. These results indicate that cholesterol infusion to the striatum can\u00a0exert a dose\u2010dependent, disease\u2010modifying effect and may be therapeutically relevant in HD.<\/jats:p>","DOI":"10.15252\/emmm.202012519","type":"journal-article","created":{"date-parts":[[2020,9,22]],"date-time":"2020-09-22T05:37:37Z","timestamp":1600753057000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":27,"title":["Striatal infusion of cholesterol promotes dose\u2010dependent behavioral benefits and exerts disease\u2010modifying effects in Huntington's disease mice"],"prefix":"10.1038","volume":"12","author":[{"given":"Giulia","family":"Birolini","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7048-9257","authenticated-orcid":false,"given":"Marta","family":"Valenza","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eleonora","family":"Di Paolo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elena","family":"Vezzoli","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesca","family":"Talpo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Claudia","family":"Maniezzi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Claudio","family":"Caccia","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Valerio","family":"Leoni","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Franco","family":"Taroni","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vittoria D","family":"Bocchi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paola","family":"Conforti","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elisa","family":"Sogne","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lara","family":"Petricca","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cristina","family":"Cariulo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Margherita","family":"Verani","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrea","family":"Caricasole","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1476-7742","authenticated-orcid":false,"given":"Andrea","family":"Falqui","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gerardo","family":"Biella","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0755-4917","authenticated-orcid":false,"given":"Elena","family":"Cattaneo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2020,9,22]]},"reference":[{"key":"BFEMMM202012519_CR1","doi-asserted-by":"crossref","first-page":"12799","DOI":"10.1074\/jbc.M601019200","volume":"281","author":"K Abildayeva","year":"2006","unstructured":"Abildayeva K, Jansen PJ, Hirsch\u2010Reinshagen V, Bloks VW, Bakker AHF, Ramaekers FCS, De Vente J, Groen AK, Wellington CL, Kuipers F et\u00a0al (2006) 24(S)\u2010hydroxycholesterol participates in a liver X receptor\u2010controlled pathway in astrocytes that regulates apolipoprotein E\u2010mediated cholesterol efflux. 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