{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,15]],"date-time":"2026-10-15T03:51:50Z","timestamp":1792036310793,"version":"4.4.0"},"reference-count":72,"publisher":"Oxford University Press (OUP)","issue":"10","license":[{"start":{"date-parts":[[2021,5,11]],"date-time":"2021-05-11T00:00:00Z","timestamp":1620691200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"funder":[{"DOI":"10.13039\/501100002426","name":"Telethon Foundation","doi-asserted-by":"publisher","award":["GGP17102"],"award-info":[{"award-number":["GGP17102"]}],"id":[{"id":"10.13039\/501100002426","id-type":"DOI","asserted-by":"publisher"}]},{"name":"NeurostemcellRepair","award":["602278, 2013\u201317"],"award-info":[{"award-number":["602278, 2013\u201317"]}]},{"name":"Department of Biosciences, University of Milan"},{"name":"Italian Ministry of Education, University and Research"},{"name":"Dipartimenti di Eccellenza Program","award":["2018\u20132022"],"award-info":[{"award-number":["2018\u20132022"]}]},{"name":"Department of Biology and Biotechnology \u201cL. Spallanzani\u201d, University of Pavia"},{"DOI":"10.13039\/501100004710","name":"Fondazione Umberto Veronesi","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100004710","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2021,11,29]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Brain cholesterol is produced mainly by astrocytes and is important for neuronal function. Its biosynthesis is severely reduced in mouse models of Huntington\u2019s disease. One possible mechanism is a diminished nuclear translocation of the transcription factor sterol regulatory element-binding protein 2 (SREBP2) and, consequently, reduced activation of SREBP2-controlled genes in the cholesterol biosynthesis pathway.<\/jats:p>\n                  <jats:p>Here we evaluated the efficacy of a gene therapy based on the unilateral intra-striatal injection of a recombinant adeno-associated virus 2\/5 (AAV2\/5) targeting astrocytes specifically and carrying the transcriptionally active N-terminal fragment of human SREBP2 (hSREBP2).<\/jats:p>\n                  <jats:p>Robust hSREBP2 expression in striatal glial cells in R6\/2 Huntington\u2019s disease mice activated the transcription of cholesterol biosynthesis pathway genes, restored synaptic transmission, reversed dopamine receptor D2 (Drd2) transcript levels decline, cleared mutant huntingtin aggregates and attenuated behavioural deficits. We conclude that glial SREBP2 participates in Huntington\u2019s disease brain pathogenesis in vivo and that AAV-based delivery of SREBP2 to astrocytes counteracts key features of the disease.<\/jats:p>","DOI":"10.1093\/brain\/awab186","type":"journal-article","created":{"date-parts":[[2021,5,7]],"date-time":"2021-05-07T10:46:27Z","timestamp":1620384387000},"page":"3175-3190","source":"Crossref","is-referenced-by-count":48,"title":["<i>SREBP2<\/i>\n                    gene therapy targeting striatal astrocytes ameliorates Huntington\u2019s disease phenotypes"],"prefix":"10.1093","volume":"144","author":[{"given":"Giulia","family":"Birolini","sequence":"first","affiliation":[{"name":"Department of Biosciences, University of Milan, 20133, Milan, Italy"},{"name":"Istituto Nazionale di Genetica Molecolare \u201cRomeo ed Enrica Invernizzi\u201d, 20122, Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gianluca","family":"Verlengia","sequence":"additional","affiliation":[{"name":"Division of Neuroscience, IRCCS San Raffaele Hospital, 20132, Milan, Italy"},{"name":"Department of BioMedical Sciences, Section of Pharmacology, University of Ferrara, 44121, Ferrara, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesca","family":"Talpo","sequence":"additional","affiliation":[{"name":"Department of Biology and Biotechnologies, University of Pavia, 27100, Pavia, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Claudia","family":"Maniezzi","sequence":"additional","affiliation":[{"name":"Department of Biology and Biotechnologies, University of Pavia, 27100, Pavia, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lorena","family":"Zentilin","sequence":"additional","affiliation":[{"name":"International Centre for Genetic Engineering and Biotechnology, ICGEB, 34149, Trieste, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mauro","family":"Giacca","sequence":"additional","affiliation":[{"name":"International Centre for Genetic Engineering and Biotechnology, ICGEB, 34149, Trieste, Italy"},{"name":"School of Cardiovascular Medicine and Sciences, King's College London, SE5 9NU, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paola","family":"Conforti","sequence":"additional","affiliation":[{"name":"Department of Biosciences, University of Milan, 20133, Milan, Italy"},{"name":"Istituto Nazionale di Genetica Molecolare \u201cRomeo ed Enrica Invernizzi\u201d, 20122, Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chiara","family":"Cordiglieri","sequence":"additional","affiliation":[{"name":"Istituto Nazionale di Genetica Molecolare \u201cRomeo ed Enrica Invernizzi\u201d, 20122, Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Claudio","family":"Caccia","sequence":"additional","affiliation":[{"name":"Unit of Medical Genetics and Neurogenetics. Fondazione IRCCS Istituto Neurologico Carlo Besta, 20131 Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Valerio","family":"Leoni","sequence":"additional","affiliation":[{"name":"School of Medicine and Surgery, University of Milano-Bicocca, 20900, Monza, Italy"},{"name":"Laboratory of Clinical Pathology, Hospital of Desio, ASST Monza, 20900, Monza, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2420-5233","authenticated-orcid":false,"given":"Franco","family":"Taroni","sequence":"additional","affiliation":[{"name":"Unit of Medical Genetics and Neurogenetics. Fondazione IRCCS Istituto Neurologico Carlo Besta, 20131 Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gerardo","family":"Biella","sequence":"additional","affiliation":[{"name":"Department of Biology and Biotechnologies, University of Pavia, 27100, Pavia, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3586-2693","authenticated-orcid":false,"given":"Michele","family":"Simonato","sequence":"additional","affiliation":[{"name":"Division of Neuroscience, IRCCS San Raffaele Hospital, 20132, Milan, Italy"},{"name":"Department of BioMedical Sciences, Section of Pharmacology, University of Ferrara, 44121, Ferrara, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elena","family":"Cattaneo","sequence":"additional","affiliation":[{"name":"Department of Biosciences, University of Milan, 20133, Milan, Italy"},{"name":"Istituto Nazionale di Genetica Molecolare \u201cRomeo ed Enrica 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