Please use this identifier to cite or link to this item:
https://hdl.handle.net/10316/108721
DC Field | Value | Language |
---|---|---|
dc.contributor.author | Cunha-Santos, Janete | - |
dc.contributor.author | Duarte-Neves, Joana | - |
dc.contributor.author | Carmona, Vitor | - |
dc.contributor.author | Guarente, Leonard | - |
dc.contributor.author | Almeida, Luís Pereira de | - |
dc.contributor.author | Cavadas, Cláudia | - |
dc.date.accessioned | 2023-09-11T08:26:25Z | - |
dc.date.available | 2023-09-11T08:26:25Z | - |
dc.date.issued | 2016-05-11 | - |
dc.identifier.issn | 2041-1723 | pt |
dc.identifier.uri | https://hdl.handle.net/10316/108721 | - |
dc.description.abstract | Machado-Joseph disease (MJD) is a neurodegenerative disorder characterized by an abnormal expansion of the CAG triplet in the ATXN3 gene, translating into a polyglutamine tract within the ataxin-3 protein. The available treatments only ameliorate symptomatology and do not block disease progression. In this study we find that caloric restriction dramatically rescues the motor incoordination, imbalance and the associated neuropathology in transgenic MJD mice. We further show that caloric restriction rescues SIRT1 levels in transgenic MJD mice, whereas silencing SIRT1 is sufficient to prevent the beneficial effects on MJD pathology. In addition, the re-establishment of SIRT1 levels in MJD mouse model, through the gene delivery approach, significantly ameliorates neuropathology, reducing neuroinflammation and activating autophagy. Furthermore, the pharmacological activation of SIRT1 with resveratrol significantly reduces motor incoordination of MJD mice. The pharmacological SIRT1 activation could provide important benefits to treat MJD patients. | pt |
dc.language.iso | eng | pt |
dc.publisher | Springer Nature | pt |
dc.relation | CENTRO-07-ST24-FEDER-002002 | pt |
dc.relation | UID/NEU/ 04539/2013 | pt |
dc.relation | E-Rare4/0003/2012 Joint call for European Research Project on Rare Diseases | pt |
dc.relation | French Muscular Dystrophy Association (AFM) | pt |
dc.relation | National Ataxia Foundation (NAF) | pt |
dc.relation | Richard Chin and Lily Lock Machado-Joseph disease Research Fund | pt |
dc.relation | SFRH/BD/87404/2012 | pt |
dc.relation | SFRH/BD/74993/2010 | pt |
dc.relation | SRFH/BD/87048/2012 | pt |
dc.rights | openAccess | pt |
dc.rights.uri | http://creativecommons.org/licenses/by/4.0/ | pt |
dc.subject.mesh | Animals | pt |
dc.subject.mesh | Ataxin-3 | pt |
dc.subject.mesh | Autophagy | pt |
dc.subject.mesh | Cell Line, Tumor | pt |
dc.subject.mesh | Cerebellum | pt |
dc.subject.mesh | Disease Models, Animal | pt |
dc.subject.mesh | Gait | pt |
dc.subject.mesh | Inflammation | pt |
dc.subject.mesh | Machado-Joseph Disease | pt |
dc.subject.mesh | Mice, Inbred C57BL | pt |
dc.subject.mesh | Mice, Transgenic | pt |
dc.subject.mesh | Mutant Proteins | pt |
dc.subject.mesh | Nervous System | pt |
dc.subject.mesh | Neurons | pt |
dc.subject.mesh | RNA, Messenger | pt |
dc.subject.mesh | Resveratrol | pt |
dc.subject.mesh | Sirtuin 1 | pt |
dc.subject.mesh | Stilbenes | pt |
dc.subject.mesh | Caloric Restriction | pt |
dc.subject.mesh | Motor Activity | pt |
dc.title | Caloric restriction blocks neuropathology and motor deficits in Machado-Joseph disease mouse models through SIRT1 pathway | pt |
dc.type | article | - |
degois.publication.firstPage | 11445 | pt |
degois.publication.issue | 1 | pt |
degois.publication.title | Nature Communications | pt |
dc.peerreviewed | yes | pt |
dc.identifier.doi | 10.1038/ncomms11445 | pt |
degois.publication.volume | 7 | pt |
dc.date.embargo | 2016-05-11 | * |
uc.date.periodoEmbargo | 0 | pt |
item.openairecristype | http://purl.org/coar/resource_type/c_18cf | - |
item.grantfulltext | open | - |
item.openairetype | article | - |
item.languageiso639-1 | en | - |
item.fulltext | Com Texto completo | - |
item.cerifentitytype | Publications | - |
crisitem.author.researchunit | CNC - Center for Neuroscience and Cell Biology | - |
crisitem.author.researchunit | CIBB - Center for Innovative Biomedicine and Biotechnology | - |
crisitem.author.researchunit | CNC - Center for Neuroscience and Cell Biology | - |
crisitem.author.researchunit | CIBB - Center for Innovative Biomedicine and Biotechnology | - |
crisitem.author.orcid | 0000-0001-5831-3307 | - |
crisitem.author.orcid | 0000-0001-8020-9266 | - |
Appears in Collections: | FFUC- Artigos em Revistas Internacionais I&D CNC - Artigos em Revistas Internacionais |
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File | Description | Size | Format | |
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Caloric restriction blocks neuropathology and motor deficits in Machado-Joseph disease mouse models through SIRT1 pathway.pdf | 2.07 MB | Adobe PDF | View/Open |
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This item is licensed under a Creative Commons License