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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) 
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 
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.
ISSN: 0305-1846
DOI: 10.1111/nan.12763
Rights: embargoedAccess
Appears in Collections:I&D CNC - Artigos em Revistas Internacionais

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