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Trehalose alleviates the phenotype of Machado–Joseph disease mouse models

2020Journal article Open access
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dc.contributor.authorSantana, Magda M.
dc.contributor.authorPaixão, Susana
dc.contributor.authorCunha-Santos, Janete
dc.contributor.authorSilva, Teresa Pereira
dc.contributor.authorTrevino-Garcia, Allyson
dc.contributor.authorGaspar, Laetitia S.
dc.contributor.authorNóbrega, Clévio
dc.contributor.authorNobre, Rui Jorge
dc.contributor.authorCavadas, Cláudia
dc.contributor.authorGreif, Hagar
dc.contributor.authorPereira de Almeida, Luís
dc.date.accessioned2020-04-30T10:41:18Z
dc.date.available2020-04-30T10:41:18Z
dc.date.issued2020
dc.description.abstractMachado-Joseph disease (MJD), also known as spinocerebellar ataxia type 3, is the most common of the dominantly inherited ataxias worldwide and is characterized by mutant ataxin-3 aggregation and neuronal degeneration. There is no treatment available to block or delay disease progression. In this work we investigated whether trehalose, a natural occurring disaccharide widely used in food and cosmetic industry, would rescue biochemical, behavioral and neuropathological features of an in vitro and of a severe MJD transgenic mouse model.pt_PT
dc.description.sponsorshipThis work was funded by BioBlast Pharma, the ERDF through the Regional Operational Program Center 2020, Competitiveness Factors Operational Program (COMPETE 2020) and National Funds through FCT (Foundation for Science and Technology) - SFRH/BD/87404/2012, BrainHealth2020 projects (CENTRO-01-0145-FEDER-000008), ViraVector (CENTRO-01-0145FEDER-022095), CortaCAGs (POCI-01-0145-FEDER-016719), SpreadSilenc‑ing POCI-01-0145-FEDER-029716 and POCI-01-0145-FEDER-007440, as well as the SynSpread, ESMI and ModelPolyQ under the EU Joint ProgramNeurodegenerative Disease Research (JPND), the last two co-funded bythe European Union H2020 program, GA No. 643417; by National Ataxia Foundation (USA), the American Portuguese Biomedical Research Fund (APBRF) and the Richard Chin and Lily Lock Machado–Joseph Disease Research Fund.pt_PT
dc.description.versioninfo:eu-repo/semantics/publishedVersionpt_PT
dc.identifier.doi10.1186/s12967-020-02302-2pt_PT
dc.identifier.issn1479-5876
dc.identifier.urihttp://hdl.handle.net/10400.1/13814
dc.language.isoengpt_PT
dc.peerreviewedyespt_PT
dc.publisherBMCpt_PT
dc.relationDELAYING NEURODEGENERATION BY CALORIC RESTRICTION APPROACHES: FROM MECHANISMS TO MOLECULAR THERAPY.
dc.rights.urihttp://creativecommons.org/licenses/by/4.0/pt_PT
dc.subjectMachado-Joseph diseasept_PT
dc.subjectAutophagypt_PT
dc.subjectSpinocerebellar ataxia type 3pt_PT
dc.subjectPolyglutamine disorderpt_PT
dc.subjectTrehalosept_PT
dc.titleTrehalose alleviates the phenotype of Machado–Joseph disease mouse modelspt_PT
dc.typejournal article
dspace.entity.typePublication
oaire.awardTitleDELAYING NEURODEGENERATION BY CALORIC RESTRICTION APPROACHES: FROM MECHANISMS TO MOLECULAR THERAPY.
oaire.awardURIinfo:eu-repo/grantAgreement/FCT//SFRH%2FBD%2F87404%2F2012/PT
oaire.citation.issue1pt_PT
oaire.citation.startPage161pt_PT
oaire.citation.titleJournal of Translational Medicinept_PT
oaire.citation.volume18pt_PT
person.familyNameNóbrega
person.givenNameClévio
person.identifier.ciencia-idC510-7F41-BAF8
person.identifier.orcid0000-0002-8312-5292
person.identifier.ridM-6047-2013
person.identifier.scopus-author-id24473454000
project.funder.identifierhttp://doi.org/10.13039/501100001871
project.funder.nameFundação para a Ciência e a Tecnologia
rcaap.rightsopenAccesspt_PT
rcaap.typearticlept_PT
relation.isAuthorOfPublication725ea6f8-1363-4cee-9cf2-5ac7303b3ba9
relation.isAuthorOfPublication.latestForDiscovery725ea6f8-1363-4cee-9cf2-5ac7303b3ba9
relation.isProjectOfPublication443614eb-5e60-4509-bd00-d9e54c6ba480
relation.isProjectOfPublication.latestForDiscovery443614eb-5e60-4509-bd00-d9e54c6ba480

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