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The cholesterol 24-hydroxylase activates autophagy and decreases mutant huntingtin build-up in a neuroblastoma culture model of Huntington’s disease

2020-04-10Journal article Open access
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dc.contributor.authorNóbrega, Clévio
dc.contributor.authorConceição, André
dc.contributor.authorCosta, Rafael G
dc.contributor.authorKoppenol, Rebekah
dc.contributor.authorSequeira, Raquel L.
dc.contributor.authorNunes, Ricardo
dc.contributor.authorCarmo-Silva, Sara
dc.contributor.authorMarcelo, Adriana
dc.contributor.authorMatos, Carlos A
dc.contributor.authorBetuing, Sandrine
dc.contributor.authorCaboche, Jocelyne
dc.contributor.authorCartier, Nathalie
dc.contributor.authorAlves, Sandro
dc.date.accessioned2020-05-08T14:21:55Z
dc.date.available2020-05-08T14:21:55Z
dc.date.issued2020-04-10
dc.date.updated2020-05-01T03:32:03Z
dc.description.abstractObjective Compromised brain cholesterol turnover and altered regulation of brain cholesterol metabolism have been allied with some neurodegenerative diseases, including Huntington’s disease (HD). Following our previous studies in HD, in this study we aim to investigate in vitro in a neuroblastoma cellular model of HD, the effect of CYP46A1 overexpression, an essential enzyme in cholesterol metabolism, on huntingtin aggregation and levels. Results We found that CYP46A1 reduces the quantity and size of mutant huntingtin aggregates in cells, as well as the levels of mutant huntingtin protein. Additionally, our results suggest that the observed beneficial effects of CYP46A1 in HD cells are linked to the activation of autophagy. Taken together, our results further demonstrate that CYP46A1 is a pertinent target to counteract HD progression.pt_PT
dc.description.sponsorshipThis work was supported by Brainvectis and E.rare: E-Rare Joint Transnational Call for Proposals 2017 “Transnational Research Projects for Innovative Therapeutic Approaches for Rare Diseases”. CN laboratory is supported by the French Muscular Dystrophy Association (AFM-Téléthon), the Ataxia UK, and the Fundação para a Ciência e Tecnologia (project ALG-01-0145-FEDER-29480 “SeGrPolyQ”). AM is supported by a Ph.D. fellowship from FCT (SFRH/BD/133192/2017)pt_PT
dc.description.versioninfo:eu-repo/semantics/publishedVersionpt_PT
dc.identifier.citationBMC Research Notes. 2020 Apr 10;13(1):210pt_PT
dc.identifier.dois13104-020-05053-xpt_PT
dc.identifier.urihttp://hdl.handle.net/10400.1/13861
dc.language.isoengpt_PT
dc.peerreviewedyespt_PT
dc.publisherBMCpt_PT
dc.rights.holderThe Author(s)
dc.rights.urihttp://creativecommons.org/licenses/by/4.0/pt_PT
dc.titleThe cholesterol 24-hydroxylase activates autophagy and decreases mutant huntingtin build-up in a neuroblastoma culture model of Huntington’s diseasept_PT
dc.typejournal article
dspace.entity.typePublication
oaire.citation.issue1pt_PT
oaire.citation.startPage210pt_PT
oaire.citation.titleBMC Research Notespt_PT
oaire.citation.volume13pt_PT
person.familyNameNóbrega
person.familyNameCavaco Koppenol
person.familyNameLourenço das Neves Sequeira
person.familyNameNunes
person.familyNameMarcelo
person.familyNameAlbuquerque Andrade de Matos
person.givenNameClévio
person.givenNameRebekah
person.givenNameRaquel
person.givenNameRicardo
person.givenNameAdriana
person.givenNameCarlos Adriano
person.identifier.ciencia-idC510-7F41-BAF8
person.identifier.ciencia-id3411-D6AA-DA14
person.identifier.ciencia-id081A-EC7D-DEEC
person.identifier.ciencia-id7613-00C2-1621
person.identifier.ciencia-idDC14-C299-222A
person.identifier.orcid0000-0002-8312-5292
person.identifier.orcid0000-0003-3239-7324
person.identifier.orcid0000-0002-0500-6300
person.identifier.orcid0000-0002-7327-0170
person.identifier.orcid0000-0002-9019-7569
person.identifier.ridM-6047-2013
person.identifier.scopus-author-id24473454000
rcaap.rightsopenAccesspt_PT
rcaap.typearticlept_PT
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