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https://hdl.handle.net/10316/96433| Title: | The autophagy-enhancing drug carbamazepine improves neuropathology and motor impairment in mouse models of Machado-Joseph disease | Authors: | Vasconcelos-Ferreira, Ana Carmo-Silva, Sara Codêsso, José Miguel Antunes Silva, Patrick Joel da Martinez, Alberto Rolim Muro França, Marcondes Cavalcante Nóbrega, Clévio David Rodrigues Almeida, Luís Pereira de |
Keywords: | Machado-Joseph disease; Ataxin-3; Autophagy; Carbamazepine; Neuroprotection | Issue Date: | 25-Aug-2021 | Publisher: | British Neuropathological Society; Wiley | Project: | CENTRO-01-0145-FEDER-000008 (BRAINHEALTH 2020) UID/NEU/04539/2020 CENTRO-01-0145-FEDER (ViraVector) CancelStem , POCI-01-0145-FEDER-032309 PTDC/NEU-NMC/0084/2014 (CortaCAGs) POCI-01-0145-FEDER-016719 (CortaCAGs) POCI-01-0145-FEDER-029716 (SpreadSilencing) POCI-01-0145-FEDER-016807 (Imagene) POCI-01-0145-FEDER-016390 (CancelStem) POCI-01-0145-FEDER-032309 (CancelStem ) info:eu-repo/grantAgreement/EC/H2020/643417/EU/ERA-NET for establishing synergies between the Joint Programming on Neurodegenerative Diseases Research and Horizon 2020 SFRH/BD/87804/2012 |
Serial title, monograph or event: | Neuropathology and Applied Neurobiology | Abstract: | Machado-Joseph disease (MJD), or spinocerebellar ataxia type 3 (SCA3), is the most common autosomal dominantly-inherited ataxia worldwide and is characterised by the accumulation of mutant ataxin-3 (mutATXN3) in different brain regions, leading to neurodegeneration. Currently, there are no available treatments able to block disease progression. In this study, we investigated whether carbamazepine (CBZ) would activate autophagy and mitigate MJD pathology. | URI: | https://hdl.handle.net/10316/96433 | ISSN: | 0305-1846 1365-2990 |
DOI: | 10.1111/nan.12763 | Rights: | embargoedAccess |
| Appears in Collections: | I&D CNC - Artigos em Revistas Internacionais |
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