The cholesterol 24-hydroxylase activates autophagy and decreases mutant huntingtin build-up in a neuroblastoma culture model of Huntington’s disease - Archive ouverte HAL
Article Dans Une Revue BMC Research Notes Année : 2020

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

Clévio Nóbrega
  • Fonction : Auteur
André Conceição
  • Fonction : Auteur
Rafael G Costa
  • Fonction : Auteur
Rebekah Koppenol
  • Fonction : Auteur
Raquel L Sequeira
  • Fonction : Auteur
Ricardo Nunes
  • Fonction : Auteur
Sara Carmo-Silva
  • Fonction : Auteur
Adriana Marcelo
  • Fonction : Auteur
Carlos A Matos
  • Fonction : Auteur
Sandrine Betuing
Nathalie Cartier
  • Fonction : Auteur
Sandro Alves
  • Fonction : Auteur

Résumé

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.

Domaines

Neurobiologie
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Dates et versions

hal-03053649 , version 1 (11-12-2020)

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Citer

Clévio Nóbrega, André Conceição, Rafael G Costa, Rebekah Koppenol, Raquel L Sequeira, et al.. The cholesterol 24-hydroxylase activates autophagy and decreases mutant huntingtin build-up in a neuroblastoma culture model of Huntington’s disease. BMC Research Notes, 2020, 13, pp.210. ⟨10.1186/s13104-020-05053-x⟩. ⟨hal-03053649⟩
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