Utilize este identificador para referenciar este registo:
https://hdl.handle.net/10316/108721
Título: | Caloric restriction blocks neuropathology and motor deficits in Machado-Joseph disease mouse models through SIRT1 pathway | Autor: | Cunha-Santos, Janete Duarte-Neves, Joana Carmona, Vitor Guarente, Leonard Almeida, Luís Pereira de Cavadas, Cláudia |
Data: | 11-Mai-2016 | Editora: | Springer Nature | Projeto: | CENTRO-07-ST24-FEDER-002002 UID/NEU/ 04539/2013 E-Rare4/0003/2012 Joint call for European Research Project on Rare Diseases French Muscular Dystrophy Association (AFM) National Ataxia Foundation (NAF) Richard Chin and Lily Lock Machado-Joseph disease Research Fund SFRH/BD/87404/2012 SFRH/BD/74993/2010 SRFH/BD/87048/2012 |
Título da revista, periódico, livro ou evento: | Nature Communications | Volume: | 7 | Número: | 1 | Resumo: | 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. | URI: | https://hdl.handle.net/10316/108721 | ISSN: | 2041-1723 | DOI: | 10.1038/ncomms11445 | Direitos: | openAccess |
Aparece nas coleções: | FFUC- Artigos em Revistas Internacionais I&D CNC - Artigos em Revistas Internacionais |
Ficheiros deste registo:
Ficheiro | Descrição | Tamanho | Formato | |
---|---|---|---|---|
Caloric restriction blocks neuropathology and motor deficits in Machado-Joseph disease mouse models through SIRT1 pathway.pdf | 2.07 MB | Adobe PDF | Ver/Abrir |
Visualizações de página
86
Visto em 28/ago/2024
Downloads
37
Visto em 28/ago/2024
Google ScholarTM
Verificar
Altmetric
Altmetric
Este registo está protegido por Licença Creative Commons