Utilize este identificador para referenciar este registo:
https://hdl.handle.net/1822/86970
Título: | 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 |
Autor(es): | Garcia, Daniela Raquel Cunha Fernandes, Daniela Monteiro Correia, Joana Sofia Carvalho, Andreia Alexandra Neves Ferreira, Ana Catarina Coutinho Vilaça Gomes, Sónia Isabel Nunes Guerra Viana, João Filipe Oliveira Oliveira, João F. Castro, Andreia Cristiana Teixeira Maciel, P. Silva, Sara Carina Duarte |
Palavras-chave: | Astrocyte Machado–Joseph disease CMVMJD135 mice IP3R2 KO mice Motor behavior Spinocerebellar ataxias IP R2 KO mice 3 |
Data: | 25-Jun-2023 |
Editora: | Multidisciplinary Digital Publishing Institute (MDPI) |
Revista: | International Journal of Molecular Sciences |
Citação: | 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 |
Resumo(s): | 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. |
Tipo: | Artigo |
URI: | https://hdl.handle.net/1822/86970 |
DOI: | 10.3390/ijms241310606 |
ISSN: | 1661-6596 |
e-ISSN: | 1422-0067 |
Versão da editora: | https://www.mdpi.com/1422-0067/24/13/10606 |
Arbitragem científica: | yes |
Acesso: | Acesso aberto |
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