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

Data de publicação: Data Ahead of Print:

Autores da FMUP

  • Rui Manuel Lopes Nunes

    Autor

Participantes de fora da FMUP

  • Nóbrega C
  • Conceição A
  • Costa RG
  • Koppenol R
  • Sequeira RL
  • Carmo-Silva S
  • Marcelo A
  • Matos CA
  • Betuing S
  • Caboche J
  • Cartier N
  • Alves S

Unidades de investigação

Abstract

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.

Dados da publicação

ISSN/ISSNe:
1756-0500, 1756-0500

BMC Research Notes  BioMed Central Ltd.

Tipo:
Article
Páginas:
210-210

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Keywords

  • Autophagy; CYP46A1; Cholesterol; Huntingtin; Neuroblastoma cells

Proyectos asociados

Ameaça aos princípios bioéticos na pratica médica no Brasil: Duas Décadas dos Processos ético-profissionais (1988-2008)

Investigador Principal: Rui Manuel Lopes Nunes

Estudo Clínico Académico . 2019

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