The autophagy-enhancing drug carbamazepine improves neuropathology and motor impairment in mouse models of Machado-Joseph disease
Autor(a) principal: | |
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Data de Publicação: | 2021 |
Outros Autores: | , , , , , , |
Tipo de documento: | Artigo |
Idioma: | eng |
Título da fonte: | Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
Texto Completo: | http://hdl.handle.net/10316/96433 https://doi.org/10.1111/nan.12763 |
Resumo: | 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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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/articlehttp://hdl.handle.net/10316/96433http://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:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãoinstacron:RCAAP2022-05-25T03:53:43Zoai:estudogeral.uc.pt:10316/96433Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireopendoar:71602024-03-19T21:14:41.164205Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãofalse |
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 |
http://hdl.handle.net/10316/96433 http://hdl.handle.net/10316/96433 https://doi.org/10.1111/nan.12763 |
url |
http://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:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação instacron:RCAAP |
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Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação |
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RCAAP |
institution |
RCAAP |
reponame_str |
Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
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Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
repository.name.fl_str_mv |
Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação |
repository.mail.fl_str_mv |
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1799134045056008192 |