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https://hdl.handle.net/10316/105836
Título: | The cholesterol 24-hydroxylase activates autophagy and decreases mutant huntingtin build-up in a neuroblastoma culture model of Huntington's disease | Autor: | Nóbrega, Clévio Conceição, André Francisco da Costa, Rafael G Koppenol, Rebekah Sequeira, Raquel L Nunes, Ricardo Carmo-Silva, Sara Marcelo, Adriana Matos, Carlos A. Betuing, Sandrine Caboche, Jocelyne Cartier, Nathalie Alves, Sandro |
Palavras-chave: | CYP46A1; Cholesterol; Neuroblastoma cells; Huntingtin; Autophagy | Data: | 10-Abr-2020 | Editora: | Springer Nature | Projeto: | Brainvectis and E.rare: E-Rare Joint Transnational Call for Proposals 2017 “Transnational Research Projects for Innovative Therapeutic Approaches for Rare Diseases”. French Muscular Dystrophy Association (AFM-Téléthon) Ataxia UK project ALG-01-0145-FEDER-29480 “SeGrPolyQ” Ph.D. fellowship from FCT (SFRH/ BD/133192/2017) |
Título da revista, periódico, livro ou evento: | BMC Research Notes | Volume: | 13 | Número: | 1 | Resumo: | Objective: 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. | URI: | https://hdl.handle.net/10316/105836 | ISSN: | 1756-0500 | DOI: | 10.1186/s13104-020-05053-x | Direitos: | openAccess |
Aparece nas coleções: | I&D CNC - Artigos em Revistas Internacionais |
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The-cholesterol-24hydroxylase-activates-autophagy-and-decreases-mutant-huntingtin-buildup-in-a-neuroblastoma-culture-model-of-Huntingtons-diseaseBMC-Research-Notes.pdf | 1.61 MB | Adobe PDF | Ver/Abrir |
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