{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,15]],"date-time":"2026-10-15T03:52:01Z","timestamp":1792036321017,"version":"4.4.0"},"reference-count":39,"publisher":"Elsevier BV","license":[{"start":{"date-parts":[[2017,2,1]],"date-time":"2017-02-01T00:00:00Z","timestamp":1485907200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"}],"funder":[{"DOI":"10.13039\/100005725","name":"CHDI Foundation","doi-asserted-by":"publisher","award":["A-5086"],"award-info":[{"award-number":["A-5086"]}],"id":[{"id":"10.13039\/100005725","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100005725","name":"CHDI Foundation","doi-asserted-by":"publisher","award":["A-5142"],"award-info":[{"award-number":["A-5142"]}],"id":[{"id":"10.13039\/100005725","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100005725","name":"CHDI Foundation","doi-asserted-by":"publisher","award":["GR-2008"],"award-info":[{"award-number":["GR-2008"]}],"id":[{"id":"10.13039\/100005725","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["clinicalkey.jp","clinicalkey.com","clinicalkey.es","clinicalkey.com.au","clinicalkey.fr","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Neurobiology of Disease"],"published-print":{"date-parts":[[2017,2]]},"DOI":"10.1016\/j.nbd.2016.11.013","type":"journal-article","created":{"date-parts":[[2016,11,30]],"date-time":"2016-11-30T09:56:16Z","timestamp":1480499776000},"page":"66-76","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":54,"special_numbering":"C","title":["Early and brain region-specific decrease of de novo cholesterol biosynthesis in Huntington's disease: A cross-validation study in Q175 knock-in mice"],"prefix":"10.1016","volume":"98","author":[{"given":"Mahalakshmi","family":"Shankaran","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eleonora","family":"Di Paolo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Valerio","family":"Leoni","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Claudio","family":"Caccia","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Costanza","family":"Ferrari Bardile","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hussein","family":"Mohammed","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stefano","family":"Di Donato","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Seung","family":"Kwak","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Deanna","family":"Marchionini","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Scott","family":"Turner","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elena","family":"Cattaneo","sequence":"additional","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"}]}],"member":"78","reference":[{"key":"10.1016\/j.nbd.2016.11.013_bb0005","doi-asserted-by":"crossref","first-page":"12799","DOI":"10.1074\/jbc.M601019200","article-title":"24(S)-hydroxycholesterol participates in a liver X receptor-controlled pathway in astrocytes that regulates apolipoprotein E-mediated cholesterol efflux","volume":"281","author":"Abildayeva","year":"2006","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.nbd.2016.11.013_bb0010","doi-asserted-by":"crossref","first-page":"585","DOI":"10.1016\/j.tcb.2011.06.004","article-title":"Central nervous system myelin: structure, synthesis and assembly","volume":"21","author":"Aggarwal","year":"2011","journal-title":"Trends Cell Biol."},{"key":"10.1016\/j.nbd.2016.11.013_bb0015","doi-asserted-by":"crossref","first-page":"15005","DOI":"10.1038\/nrdp.2015.5","article-title":"Huntington disease","volume":"1","author":"Bates","year":"2015","journal-title":"Nature Rev. Dis. Primers"},{"key":"10.1016\/j.nbd.2016.11.013_bb0020","doi-asserted-by":"crossref","first-page":"1594","DOI":"10.1016\/S0022-2275(20)32188-X","article-title":"Cholesterol homeostasis in human brain: turnover of 24S-hydroxycholesterol and evidence for a cerebral origin of most of this oxysterol in the circulation","volume":"39","author":"Bjorkhem","year":"1998","journal-title":"J. Lipid Res."},{"key":"10.1016\/j.nbd.2016.11.013_bb0025","doi-asserted-by":"crossref","first-page":"953","DOI":"10.1093\/brain\/awv384","article-title":"CYP46A1, the rate-limiting enzyme for cholesterol degradation, is neuroprotective in Huntington's disease","volume":"139","author":"Boussicault","year":"2016","journal-title":"Brain J. Neurol."},{"key":"10.1016\/j.nbd.2016.11.013_bb0030","doi-asserted-by":"crossref","first-page":"628","DOI":"10.1111\/j.1742-4658.2008.06808.x","article-title":"SREBPs: SREBP function in glia-neuron interactions","volume":"276","author":"Camargo","year":"2009","journal-title":"FEBS J."},{"key":"10.1016\/j.nbd.2016.11.013_bb0035","doi-asserted-by":"crossref","first-page":"854","DOI":"10.1093\/clinchem\/26.7.854","article-title":"Total serum cholesterol by isotope dilution\/mass spectrometry: a candidate definitive method","volume":"26","author":"Cohen","year":"1980","journal-title":"Clin. Chem."},{"key":"10.1016\/j.nbd.2016.11.013_bb0040","doi-asserted-by":"crossref","first-page":"338","DOI":"10.1007\/BF01716251","article-title":"Regulation of cholesterol metabolism in man and in other species","volume":"62","author":"Dietschy","year":"1984","journal-title":"Klin. Wochenschr."},{"key":"10.1016\/j.nbd.2016.11.013_bb0045","doi-asserted-by":"crossref","first-page":"1375","DOI":"10.1194\/jlr.R400004-JLR200","article-title":"Thematic review series: brain lipids. Cholesterol metabolism in the central nervous system during early development and in the mature animal","volume":"45","author":"Dietschy","year":"2004","journal-title":"J. Lipid Res."},{"key":"10.1016\/j.nbd.2016.11.013_bb0050","first-page":"E988","article-title":"Mass isotopomer distribution analysis: a technique for measuring biosynthesis and turnover of polymers","volume":"263","author":"Hellerstein","year":"1992","journal-title":"Am. J. Phys."},{"key":"10.1016\/j.nbd.2016.11.013_bb0055","doi-asserted-by":"crossref","first-page":"965","DOI":"10.1093\/hmg\/ddl013","article-title":"Regional and cellular gene expression changes in human Huntington's disease brain","volume":"15","author":"Hodges","year":"2006","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.nbd.2016.11.013_bb0060","doi-asserted-by":"crossref","first-page":"65","DOI":"10.1016\/j.tem.2011.10.004","article-title":"SREBPs: metabolic integrators in physiology and metabolism","volume":"23","author":"Jeon","year":"2012","journal-title":"Trends Endocrinol. Metab."},{"key":"10.1016\/j.nbd.2016.11.013_bb0065","doi-asserted-by":"crossref","first-page":"895","DOI":"10.1046\/j.1471-4159.1995.64020895.x","article-title":"Cholesterol for synthesis of myelin is made locally, not imported into brain","volume":"64","author":"Jurevics","year":"1995","journal-title":"J. Neurochem."},{"key":"10.1016\/j.nbd.2016.11.013_bb0070","doi-asserted-by":"crossref","first-page":"561","DOI":"10.1038\/nrneurol.2011.132","article-title":"Cholesterol metabolism in Huntington disease","volume":"7","author":"Karasinska","year":"2011","journal-title":"Nat. Rev. Neurol."},{"key":"10.1016\/j.nbd.2016.11.013_bb0075","doi-asserted-by":"crossref","first-page":"1149","DOI":"10.1016\/S0022-2275(20)38456-X","article-title":"Serum lathosterol concentration is an indicator of whole-body cholesterol synthesis in humans","volume":"29","author":"Kempen","year":"1988","journal-title":"J. Lipid Res."},{"key":"10.1016\/j.nbd.2016.11.013_bb0080","first-page":"E372","article-title":"Measurement of fractional lipid synthesis using deuterated water (2H2O) and mass isotopomer analysis","volume":"266","author":"Lee","year":"1994","journal-title":"Am. J. Phys."},{"key":"10.1016\/j.nbd.2016.11.013_bb0085","doi-asserted-by":"crossref","first-page":"303","DOI":"10.1016\/j.neuron.2014.12.019","article-title":"Reinstating aberrant mTORC1 activity in Huntington's disease mice improves disease phenotypes","volume":"85","author":"Lee","year":"2015","journal-title":"Neuron"},{"key":"10.1016\/j.nbd.2016.11.013_bb0090","doi-asserted-by":"crossref","first-page":"2851","DOI":"10.1093\/brain\/awn212","article-title":"Plasma 24S-hydroxycholesterol and caudate MRI in pre-manifest and early Huntington's disease","volume":"131","author":"Leoni","year":"2008","journal-title":"Brain J. Neurol."},{"key":"10.1016\/j.nbd.2016.11.013_bb0095","doi-asserted-by":"crossref","first-page":"245","DOI":"10.1016\/j.neulet.2011.03.025","article-title":"Whole body cholesterol metabolism is impaired in Huntington's disease","volume":"494","author":"Leoni","year":"2011","journal-title":"Neurosci. Lett."},{"key":"10.1016\/j.nbd.2016.11.013_bb0100","doi-asserted-by":"crossref","first-page":"37","DOI":"10.1016\/j.nbd.2013.03.013","article-title":"Plasma 24S-hydroxycholesterol correlation with markers of Huntington disease progression","volume":"55","author":"Leoni","year":"2013","journal-title":"Neurobiol. Dis."},{"key":"10.1016\/j.nbd.2016.11.013_bb0105","doi-asserted-by":"crossref","first-page":"7238","DOI":"10.1073\/pnas.96.13.7238","article-title":"cDNA cloning of cholesterol 24-hydroxylase, a mediator of cholesterol homeostasis in the brain","volume":"96","author":"Lund","year":"1999","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.nbd.2016.11.013_bb0110","doi-asserted-by":"crossref","first-page":"9799","DOI":"10.1073\/pnas.93.18.9799","article-title":"Cholesterol homeostasis in human brain: evidence for an age-dependent flux of 24S-hydroxycholesterol from the brain into the circulation","volume":"93","author":"Lutjohann","year":"1996","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.nbd.2016.11.013_bb0115","doi-asserted-by":"crossref","first-page":"1354","DOI":"10.1126\/science.294.5545.1354","article-title":"CNS synaptogenesis promoted by glia-derived cholesterol","volume":"294","author":"Mauch","year":"2001","journal-title":"Science"},{"key":"10.1016\/j.nbd.2016.11.013_bb0120","doi-asserted-by":"crossref","first-page":"293","DOI":"10.1016\/S1388-1981(00)00070-6","article-title":"Formation of oxysterols from different pools of cholesterol as studied by stable isotope technique: cerebral origin of most circulating 24S-hydroxycholesterol in rats, but not in mice","volume":"1486","author":"Meaney","year":"2000","journal-title":"Biochim. Biophys. Acta"},{"key":"10.1016\/j.nbd.2016.11.013_bb0125","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0049838","article-title":"Comprehensive behavioral and molecular characterization of a new knock-in mouse model of Huntington's disease: zQ175","volume":"7","author":"Menalled","year":"2012","journal-title":"PLoS One"},{"key":"10.1016\/j.nbd.2016.11.013_bb0130","doi-asserted-by":"crossref","first-page":"125","DOI":"10.1111\/j.1471-4159.2009.05917.x","article-title":"Marked differences in cholesterol synthesis between neurons and glial cells from postnatal rats","volume":"109","author":"Nieweg","year":"2009","journal-title":"J. Neurochem."},{"key":"10.1016\/j.nbd.2016.11.013_bb0135","doi-asserted-by":"crossref","first-page":"1676","DOI":"10.1002\/cne.21605","article-title":"Neuronal expression and subcellular localization of cholesterol 24-hydroxylase in the mouse brain","volume":"507","author":"Ramirez","year":"2008","journal-title":"J. Comp. Neurol."},{"key":"10.1016\/j.nbd.2016.11.013_bb0140","doi-asserted-by":"crossref","first-page":"204","DOI":"10.1038\/nrneurol.2014.24","article-title":"Huntington disease: natural history, biomarkers and prospects for therapeutics","volume":"10","author":"Ross","year":"2014","journal-title":"Nat. Rev. Neurol."},{"key":"10.1016\/j.nbd.2016.11.013_bb0145","doi-asserted-by":"crossref","first-page":"2299","DOI":"10.1046\/j.1471-4159.1995.65052299.x","article-title":"Distribution of branch point prenyltransferases in regions of bovine brain","volume":"65","author":"Runquist","year":"1995","journal-title":"J. Neurochem."},{"key":"10.1016\/j.nbd.2016.11.013_bb0150","doi-asserted-by":"crossref","first-page":"489","DOI":"10.1007\/978-90-481-8622-8_18","article-title":"Cholesterol and myelin biogenesis","volume":"51","author":"Saher","year":"2010","journal-title":"Subcell. Biochem."},{"key":"10.1016\/j.nbd.2016.11.013_bb0155","doi-asserted-by":"crossref","first-page":"474","DOI":"10.1016\/j.tins.2011.06.005","article-title":"Emerging roles for cholesterol in Huntington's disease","volume":"34","author":"Valenza","year":"2011","journal-title":"Trends Neurosci."},{"key":"10.1016\/j.nbd.2016.11.013_bb0160","doi-asserted-by":"crossref","first-page":"9932","DOI":"10.1523\/JNEUROSCI.3355-05.2005","article-title":"Dysfunction of the cholesterol biosynthetic pathway in Huntington's disease","volume":"25","author":"Valenza","year":"2005","journal-title":"J. Neurosci."},{"key":"10.1016\/j.nbd.2016.11.013_bb0165","doi-asserted-by":"crossref","first-page":"133","DOI":"10.1016\/j.nbd.2007.07.004","article-title":"Progressive dysfunction of the cholesterol biosynthesis pathway in the R6\/2 mouse model of Huntington's disease","volume":"28","author":"Valenza","year":"2007","journal-title":"Neurobiol. Dis."},{"key":"10.1016\/j.nbd.2016.11.013_bb0170","doi-asserted-by":"crossref","first-page":"2187","DOI":"10.1093\/hmg\/ddm170","article-title":"Cholesterol biosynthesis pathway is disturbed in YAC128 mice and is modulated by huntingtin mutation","volume":"16","author":"Valenza","year":"2007","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.nbd.2016.11.013_bb0175","doi-asserted-by":"crossref","first-page":"10844","DOI":"10.1523\/JNEUROSCI.0917-10.2010","article-title":"Cholesterol defect is marked across multiple rodent models of Huntington's disease and is manifest in astrocytes","volume":"30","author":"Valenza","year":"2010","journal-title":"J. Neurosci."},{"key":"10.1016\/j.nbd.2016.11.013_bb0180","doi-asserted-by":"crossref","first-page":"690","DOI":"10.1038\/cdd.2014.162","article-title":"Disruption of astrocyte-neuron cholesterol cross talk affects neuronal function in Huntington's disease","volume":"22","author":"Valenza","year":"2015","journal-title":"Cell Death Differ."},{"key":"10.1016\/j.nbd.2016.11.013_bb0185","doi-asserted-by":"crossref","first-page":"1547","DOI":"10.15252\/emmm.201505413","article-title":"Cholesterol-loaded nanoparticles ameliorate synaptic and cognitive function in Huntington's disease mice","volume":"7","author":"Valenza","year":"2015","journal-title":"EMBO Mol. Med."},{"key":"10.1016\/j.nbd.2016.11.013_bb0190","doi-asserted-by":"crossref","first-page":"869","DOI":"10.1016\/S0197-4580(96)00076-0","article-title":"The lipid compositions of different regions of rat brain during development and aging","volume":"17","author":"Zhang","year":"1996","journal-title":"Neurobiol. Aging"},{"key":"10.1016\/j.nbd.2016.11.013_bb0195","doi-asserted-by":"crossref","first-page":"9741","DOI":"10.1523\/JNEUROSCI.0458-08.2008","article-title":"Absence of 2-hydroxylated sphingolipids is compatible with normal neural development but causes late-onset axon and myelin sheath degeneration","volume":"28","author":"Zoller","year":"2008","journal-title":"J. Neurosci."}],"container-title":["Neurobiology of Disease"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0969996116302819?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0969996116302819?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2022,7,15]],"date-time":"2022-07-15T16:49:16Z","timestamp":1657903756000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0969996116302819"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017,2]]},"references-count":39,"alternative-id":["S0969996116302819"],"URL":"https:\/\/doi.org\/10.1016\/j.nbd.2016.11.013","relation":{},"ISSN":["0969-9961"],"issn-type":[{"value":"0969-9961","type":"print"}],"subject":[],"published":{"date-parts":[[2017,2]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Early and brain region-specific decrease of de novo cholesterol biosynthesis in Huntington's disease: A cross-validation study in Q175 knock-in mice","name":"articletitle","label":"Article Title"},{"value":"Neurobiology of Disease","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.nbd.2016.11.013","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2016 Published by Elsevier Inc.","name":"copyright","label":"Copyright"}]}}