The cholesterol 24-hydroxylase activates autophagy and decreases mutant huntingtin build-up in a neuroblastoma culture model of Huntington's disease.
Autores da FMUP
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
- Tipo:
- Article
- Páginas:
- 210-210
BMC Research Notes BioMed Central Ltd.
Documentos
- Não há documentos
Filiações
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
Citar a publicação
Nóbrega C,Conceição A,Costa RG,Koppenol R,Sequeira RL,Nunes R,Carmo S,Marcelo A,Matos CA,Betuing S,Caboche J,Cartier N,Alves S. The cholesterol 24-hydroxylase activates autophagy and decreases mutant huntingtin build-up in a neuroblastoma culture model of Huntington's disease. BMC Res. Notes. 2020. 13. (1):p. 210-210.