Cargando…

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

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 c...

Descripción completa

Detalles Bibliográficos
Autores principales:	Nóbrega, Clévio, Conceição, André, 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
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	BioMed Central 2020
Materias:	Research Note
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7149904/ https://www.ncbi.nlm.nih.gov/pubmed/32276655 http://dx.doi.org/10.1186/s13104-020-05053-x

Ejemplares similares

Altered Cholesterol Homeostasis in Huntington’s Disease
por: Kacher, Radhia, et al.
Publicado: (2022)

Multiple Aspects of Gene Dysregulation in Huntington’s Disease
por: Moumné, Lara, et al.
Publicado: (2013)

CYP46A1, the rate-limiting enzyme for cholesterol degradation, is neuroprotective in Huntington’s disease
por: Boussicault, Lydie, et al.
Publicado: (2016)

Huntington’s Disease and Striatal Signaling
por: Roze, Emmanuel, et al.
Publicado: (2011)

On the role of RNA binding proteins in polyglutamine diseases: from pathogenesis to therapeutics
por: Conceição, André, et al.
Publicado: (2023)

Dopamine D2 Receptor Stimulation Potentiates PolyQ-Huntingtin-Induced Mouse Striatal Neuron Dysfunctions via Rho/ROCK-II Activation
por: Deyts, Carole, et al.
Publicado: (2009)

MSGP: the first database of the protein components of the mammalian stress granules
por: Nunes, Catarina, et al.
Publicado: (2019)

Mitogen- and stress-activated protein kinase 1-induced neuroprotection in Huntington's disease: role on chromatin remodeling at the PGC-1-alpha promoter
por: Martin, Elodie, et al.
Publicado: (2011)

Stress granules, RNA-binding proteins and polyglutamine diseases: too much aggregation?
por: Marcelo, Adriana, et al.
Publicado: (2021)

Huntingtin Aggregates in the Olfactory Bulb in Huntington’s Disease
por: Highet, Blake, et al.
Publicado: (2020)

Cell-Type Specific Regulation of Cholesterogenesis by CYP46A1 Re-Expression in zQ175 HD Mouse Striatum
por: Pinchaud, Katleen, et al.
Publicado: (2023)

Emerging Therapies for Huntington’s Disease – Focus on N-Terminal Huntingtin and Huntingtin Exon 1
por: van der Bent, M Leontien, et al.
Publicado: (2022)

Phosphorylation of huntingtin at residue T3 is decreased in Huntington’s disease and modulates mutant huntingtin protein conformation
por: Cariulo, Cristina, et al.
Publicado: (2017)

Making (anti-) sense out of huntingtin levels in Huntington disease
por: Evers, Melvin M, et al.
Publicado: (2015)

Huntington's disease cerebrospinal fluid seeds aggregation of mutant huntingtin
por: Tan, Z, et al.
Publicado: (2015)

Salivary levels of total huntingtin are elevated in Huntington’s disease patients
por: Corey-Bloom, Jody, et al.
Publicado: (2018)

Recent Trends in Detection of Huntingtin and Preclinical Models of Huntington's Disease
por: Mantha, Neelima, et al.
Publicado: (2014)

Editorial: Cholesterol and Neurodegenerative Diseases - Pressing Questions and How to Address Them
por: Betuing, Sandrine, et al.
Publicado: (2022)

Mutant Ataxin-2 Expression in Aged Animals Aggravates Neuropathological Features Associated with Spinocerebellar Ataxia Type 2
por: Afonso, Inês T., et al.
Publicado: (2022)

Plasma dopamine-beta-hydroxylase in neuroblastoma.
por: Schöni, M., et al.
Publicado: (1984)

Huntington’s Disease: From Mutant Huntingtin Protein to Neurotrophic Factor Therapy
por: Sari, Youssef
Publicado: (2011)

Embryonic Mutant Huntingtin Aggregate Formation in Mouse Models of Huntington’s Disease
por: Osmand, Alexander P., et al.
Publicado: (2016)

A Fresh Look at Huntingtin mRNA Processing in Huntington’s Disease
por: Romo, Lindsay, et al.
Publicado: (2018)

Rac1 Activity Is Modulated by Huntingtin and Dysregulated in Models of Huntington’s Disease
por: Tousley, Adelaide, et al.
Publicado: (2019)

Polyglutamine expansion affects huntingtin conformation in multiple Huntington’s disease models
por: Daldin, Manuel, et al.
Publicado: (2017)

Huntington’s Disease Protein Huntingtin Associates with its own mRNA
por: Culver, Brady P., et al.
Publicado: (2016)

Analysis of mutant and total huntingtin expression in Huntington’s disease murine models
por: Fodale, Valentina, et al.
Publicado: (2020)

Soluble mutant huntingtin drives early human pathogenesis in Huntington’s disease
por: Miguez, Andrés, et al.
Publicado: (2023)

Salivary Huntingtin protein is uniquely associated with clinical features of Huntington’s disease
por: Parkin, Georgia M., et al.
Publicado: (2023)

Editorial: Gene Silencing and Editing Strategies for Neurodegenerative Diseases
por: Nóbrega, Clévio, et al.
Publicado: (2018)

Ultrasensitive measurement of huntingtin protein in cerebrospinal fluid demonstrates increase with Huntington disease stage and decrease following brain huntingtin suppression
por: Southwell, Amber L., et al.
Publicado: (2015)

The distribution and density of Huntingtin inclusions across the Huntington disease neocortex: regional correlations with Huntingtin repeat expansion independent of pathologic grade
por: Hickman, Richard A., et al.
Publicado: (2022)

Mutant Huntingtin Does Not Affect the Intrinsic Phenotype of Human Huntington’s Disease T Lymphocytes
por: Miller, James R. C., et al.
Publicado: (2015)

Genistein induces degradation of mutant huntingtin in fibroblasts from Huntington’s disease patients
por: Pierzynowska, Karolina, et al.
Publicado: (2019)

Disease-related Huntingtin seeding activities in cerebrospinal fluids of Huntington’s disease patients
por: Lee, C. Y. Daniel, et al.
Publicado: (2020)

Hypothalamic expression of huntingtin causes distinct metabolic changes in Huntington's disease mice
por: Dickson, Elna, et al.
Publicado: (2022)

Polyglutamine Expansion in Huntingtin and Mechanism of DNA Damage Repair Defects in Huntington’s Disease
por: Pradhan, Subrata, et al.
Publicado: (2022)

Serum dopamine beta hydroxylase in children with neuroblastoma.
por: Eldeeb, B. B., et al.
Publicado: (1983)

PolyQ Database—an integrated database on polyglutamine diseases
por: Estevam, Bernardo, et al.
Publicado: (2023)

Neuronal Targets of Mutant Huntingtin Genetic Reduction to Ameliorate Huntington’s Disease Pathogenesis in Mice
por: Wang, Nan, et al.
Publicado: (2014)

Cannot write session to /tmp/vufind_sessions/sess_aaj8regg6je2rc76m6ec4sveri