The autophagy-enhancing drug carbamazepine improves neuropathology and motor impairment in mouse models of Machado-Joseph disease

Bibliographic Details
Main Author: Vasconcelos-Ferreira, Ana
Publication Date: 2021
Other Authors: 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
Format: Article
Language: eng
Source: Repositórios Científicos de Acesso Aberto de Portugal (RCAAP)
Download full: https://hdl.handle.net/10316/96433
https://doi.org/10.1111/nan.12763
Summary: 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.
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spelling The autophagy-enhancing drug carbamazepine improves neuropathology and motor impairment in mouse models of Machado-Joseph diseaseMachado-Joseph diseaseAtaxin-3AutophagyCarbamazepineNeuroprotectionMachado-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.Fundação para a Ciência e Tecnologia; RichardChin and Lily Lock Machado-Joseph DiseaseResearch Fund; American PortugueseBiomedical Research Fund (APBRF); NationalAtaxia Foundation (USA); European UnionH2020 program, GA No.643417; EU JointProgram - Neurodegenerative DiseaseResearch (JPND); Competitiveness FactorsOperational Program (COMPETE 2020); ERDFthrough the Regional Operational ProgramCenter 2020British Neuropathological Society; Wiley2021-08-25info:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articlehttps://hdl.handle.net/10316/96433https://hdl.handle.net/10316/96433https://doi.org/10.1111/nan.12763eng0305-18461365-2990https://onlinelibrary.wiley.com/doi/epdf/10.1111/nan.12763Vasconcelos-Ferreira, AnaCarmo-Silva, SaraCodêsso, José Miguel AntunesSilva, Patrick Joel daMartinez, Alberto Rolim MuroFrança, Marcondes CavalcanteNóbrega, Clévio David RodriguesAlmeida, Luís Pereira deinfo:eu-repo/semantics/openAccessreponame:Repositórios Científicos de Acesso Aberto de Portugal (RCAAP)instname:FCCN, serviços digitais da FCT – Fundação para a Ciência e a Tecnologiainstacron:RCAAP2024-09-05T12:51:07Zoai:estudogeral.uc.pt:10316/96433Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireinfo@rcaap.ptopendoar:https://opendoar.ac.uk/repository/71602025-05-29T05:44:45.075665Repositórios Científicos de Acesso Aberto de Portugal (RCAAP) - FCCN, serviços digitais da FCT – Fundação para a Ciência e a Tecnologiafalse
dc.title.none.fl_str_mv The autophagy-enhancing drug carbamazepine improves neuropathology and motor impairment in mouse models of Machado-Joseph disease
title The autophagy-enhancing drug carbamazepine improves neuropathology and motor impairment in mouse models of Machado-Joseph disease
spellingShingle The autophagy-enhancing drug carbamazepine improves neuropathology and motor impairment in mouse models of Machado-Joseph disease
Vasconcelos-Ferreira, Ana
Machado-Joseph disease
Ataxin-3
Autophagy
Carbamazepine
Neuroprotection
title_short The autophagy-enhancing drug carbamazepine improves neuropathology and motor impairment in mouse models of Machado-Joseph disease
title_full The autophagy-enhancing drug carbamazepine improves neuropathology and motor impairment in mouse models of Machado-Joseph disease
title_fullStr The autophagy-enhancing drug carbamazepine improves neuropathology and motor impairment in mouse models of Machado-Joseph disease
title_full_unstemmed The autophagy-enhancing drug carbamazepine improves neuropathology and motor impairment in mouse models of Machado-Joseph disease
title_sort The autophagy-enhancing drug carbamazepine improves neuropathology and motor impairment in mouse models of Machado-Joseph disease
author Vasconcelos-Ferreira, Ana
author_facet 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
author_role author
author2 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
author2_role author
author
author
author
author
author
author
dc.contributor.author.fl_str_mv 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
dc.subject.por.fl_str_mv Machado-Joseph disease
Ataxin-3
Autophagy
Carbamazepine
Neuroprotection
topic Machado-Joseph disease
Ataxin-3
Autophagy
Carbamazepine
Neuroprotection
description 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.
publishDate 2021
dc.date.none.fl_str_mv 2021-08-25
dc.type.status.fl_str_mv info:eu-repo/semantics/publishedVersion
dc.type.driver.fl_str_mv info:eu-repo/semantics/article
format article
status_str publishedVersion
dc.identifier.uri.fl_str_mv https://hdl.handle.net/10316/96433
https://hdl.handle.net/10316/96433
https://doi.org/10.1111/nan.12763
url https://hdl.handle.net/10316/96433
https://doi.org/10.1111/nan.12763
dc.language.iso.fl_str_mv eng
language eng
dc.relation.none.fl_str_mv 0305-1846
1365-2990
https://onlinelibrary.wiley.com/doi/epdf/10.1111/nan.12763
dc.rights.driver.fl_str_mv info:eu-repo/semantics/openAccess
eu_rights_str_mv openAccess
dc.publisher.none.fl_str_mv British Neuropathological Society; Wiley
publisher.none.fl_str_mv British Neuropathological Society; Wiley
dc.source.none.fl_str_mv reponame:Repositórios Científicos de Acesso Aberto de Portugal (RCAAP)
instname:FCCN, serviços digitais da FCT – Fundação para a Ciência e a Tecnologia
instacron:RCAAP
instname_str FCCN, serviços digitais da FCT – Fundação para a Ciência e a Tecnologia
instacron_str RCAAP
institution RCAAP
reponame_str Repositórios Científicos de Acesso Aberto de Portugal (RCAAP)
collection Repositórios Científicos de Acesso Aberto de Portugal (RCAAP)
repository.name.fl_str_mv Repositórios Científicos de Acesso Aberto de Portugal (RCAAP) - FCCN, serviços digitais da FCT – Fundação para a Ciência e a Tecnologia
repository.mail.fl_str_mv info@rcaap.pt
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