Utilize este identificador para referenciar este registo:
https://hdl.handle.net/1822/86970
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Campo DC | Valor | Idioma |
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dc.contributor.author | Garcia, Daniela Raquel Cunha | por |
dc.contributor.author | Fernandes, Daniela Monteiro | por |
dc.contributor.author | Correia, Joana Sofia | por |
dc.contributor.author | Carvalho, Andreia Alexandra Neves | por |
dc.contributor.author | Ferreira, Ana Catarina Coutinho Vilaça | por |
dc.contributor.author | Gomes, Sónia Isabel Nunes Guerra | por |
dc.contributor.author | Viana, João Filipe Oliveira | por |
dc.contributor.author | Oliveira, João F. | por |
dc.contributor.author | Castro, Andreia Cristiana Teixeira | por |
dc.contributor.author | Maciel, P. | por |
dc.contributor.author | Silva, Sara Carina Duarte | por |
dc.date.accessioned | 2023-10-18T09:40:53Z | - |
dc.date.available | 2023-10-18T09:40:53Z | - |
dc.date.issued | 2023-06-25 | - |
dc.identifier.citation | Cunha-Garcia, D.; Monteiro-Fernandes, D.; Correia, J.S.; Neves-Carvalho, A.; Vilaça-Ferreira, A.C.; Guerra-Gomes, S.; Viana, J.F.; Oliveira, J.F.; Teixeira-Castro, A.; Maciel, P.; et al. Genetic Ablation of Inositol 1,4,5-Trisphosphate Receptor Type 2 (IP3R2) Fails to Modify Disease Progression in a Mouse Model of Spinocerebellar Ataxia Type 3. Int. J. Mol. Sci. 2023, 24, 10606. https://doi.org/10.3390/ijms241310606 | por |
dc.identifier.issn | 1661-6596 | por |
dc.identifier.uri | https://hdl.handle.net/1822/86970 | - |
dc.description.abstract | Spinocerebellar ataxia type 3 (SCA3) is a rare neurodegenerative disease caused by an abnormal polyglutamine expansion within the ataxin-3 protein (ATXN3). This leads to neurodegeneration of specific brain and spinal cord regions, resulting in a progressive loss of motor function. Despite neuronal death, non-neuronal cells, including astrocytes, are also involved in SCA3 pathogenesis. Astrogliosis is a common pathological feature in SCA3 patients and animal models of the disease. However, the contribution of astrocytes to SCA3 is not clearly defined. Inositol 1,4,5-trisphosphate receptor type 2 (IP3R2) is the predominant IP3R in mediating astrocyte somatic calcium signals, and genetically ablation of IP3R2 has been widely used to study astrocyte function. Here, we aimed to investigate the relevance of IP3R2 in the onset and progression of SCA3. For this, we tested whether IP3R2 depletion and the consecutive suppression of global astrocytic calcium signalling would lead to marked changes in the behavioral phenotype of a SCA3 mouse model, the CMVMJD135 transgenic line. This was achieved by crossing IP3R2 null mice with the CMVMJD135 mouse model and performing a longitudinal behavioral characterization of these mice using well-established motor-related function tests. Our results demonstrate that IP3R2 deletion in astrocytes does not modify SCA3 progression. | por |
dc.description.sponsorship | This work has been funded by National funds, through the Foundation for Science and Technology (FCT)—project UIDB/50026/2020 and UIDP/50026/2020, PTDC/NEUNMC/3648/2014 and COMPETE-FEDER (POCI-01-0145-FEDER-016818); fellowships to DCG (2021.08121.BD), DMF (SFRH/BD/147947/2019), JSC (SFRH/BD/140624/2018), ANC (SFRH/BPD/118779/2016), AVF (UMINHO/BIL-CNCG/2022/11), SGG (SFRH/BD/101298/2014), and JFV (2020.05109.BD); FCT Scientific Employment Stimulus (CEEC)—Individual Call position to SDS (CEECIND/00685/2020); grants from the Bial Foundation (037/18) and “the la Caixa” Foundation (LCF/PR/HR21/52410024) to JFO; and by the projects NORTE-01-0145-FEDER-000013 and NORTE-01-0145-FEDER-000023, supported by the Norte Portugal Regional Operational Programme (NORTE 2020), under the PORTUGAL 2020 Partnership Agreement, through the European Regional Development Fund (ERDF). It was also supported by grants from the ICVS Scientific Microscopy Platform, a member of the national infrastructure PPBI—Portuguese Platform of Bioimaging (PPBI-POCI-01-0145-FEDER-022122 and national funds through the Foundation for Science and Technology (FCT). | por |
dc.language.iso | eng | por |
dc.publisher | Multidisciplinary Digital Publishing Institute (MDPI) | por |
dc.relation | info:eu-repo/grantAgreement/FCT/6817 - DCRRNI ID/UIDB%2F50026%2F2020/PT | por |
dc.relation | info:eu-repo/grantAgreement/FCT/6817 - DCRRNI ID/UIDP%2F50026%2F2020/PT | por |
dc.relation | info:eu-repo/grantAgreement/FCT/9471 - RIDTI/PTDC%2FNEU-NMC%2F3648%2F2014/PT | por |
dc.relation | POCI-01-0145-FEDER-016818 | por |
dc.relation | 2021.08121.BD | por |
dc.relation | info:eu-repo/grantAgreement/FCT/POR_NORTE/SFRH%2FBD%2F147947%2F2019/PT | por |
dc.relation | info:eu-repo/grantAgreement/FCT/POR_NORTE/SFRH%2FBD%2F140624%2F2018/PT | por |
dc.relation | info:eu-repo/grantAgreement/FCT/POR_NORTE/SFRH%2FBPD%2F118779%2F2016/PT | por |
dc.relation | UMINHO/BIL-CNCG/2022/11 | por |
dc.relation | info:eu-repo/grantAgreement/FCT/POR_NORTE/SFRH%2FBD%2F101298%2F2014/PT | por |
dc.relation | info:eu-repo/grantAgreement/FCT/POR_NORTE/2020.05109.BD/PT | por |
dc.relation | CEECIND/00685/2020 | por |
dc.relation | LCF/PR/HR21/52410024 | por |
dc.relation | NORTE-01-0145-FEDER-000013 | por |
dc.relation | NORTE-01-0145-FEDER-000023 | por |
dc.relation | PPBI-POCI-01-0145-FEDER-022122 | por |
dc.rights | openAccess | por |
dc.rights.uri | http://creativecommons.org/licenses/by/4.0/ | por |
dc.subject | Astrocyte | por |
dc.subject | Machado–Joseph disease | por |
dc.subject | CMVMJD135 mice | por |
dc.subject | IP3R2 KO mice | por |
dc.subject | Motor behavior | por |
dc.subject | Spinocerebellar ataxias | por |
dc.subject | IP R2 KO mice 3 | por |
dc.title | Genetic ablation of inositol 1,4,5-Trisphosphate receptor type 2 (IP3R2) fails to modify disease progression in a mouse model of Spinocerebellar Ataxia type 3 | por |
dc.type | article | por |
dc.peerreviewed | yes | por |
dc.relation.publisherversion | https://www.mdpi.com/1422-0067/24/13/10606 | por |
oaire.citationStartPage | 1 | por |
oaire.citationEndPage | 22 | por |
oaire.citationIssue | 13 | por |
oaire.citationVolume | 24 | por |
dc.date.updated | 2023-07-13T14:07:31Z | - |
dc.identifier.eissn | 1422-0067 | - |
dc.identifier.doi | 10.3390/ijms241310606 | por |
dc.identifier.pmid | 37445783 | por |
sdum.journal | International Journal of Molecular Sciences | por |
oaire.version | VoR | por |
Aparece nas coleções: | ICVS - Artigos em revistas internacionais / Papers in international journals |
Ficheiros deste registo:
Ficheiro | Descrição | Tamanho | Formato | |
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ijms-24-10606-v2.pdf | 3,7 MB | Adobe PDF | Ver/Abrir |
Este trabalho está licenciado sob uma Licença Creative Commons