Please use this identifier to cite or link to this item:
http://hdl.handle.net/2445/183269
Full metadata record
DC Field | Value | Language |
---|---|---|
dc.contributor.author | Giralt Torroella, Albert | - |
dc.contributor.author | Saavedra, Ana | - |
dc.contributor.author | Alberch i Vié, Jordi, 1959- | - |
dc.contributor.author | Pérez Navarro, Esther | - |
dc.date.accessioned | 2022-02-17T19:17:59Z | - |
dc.date.available | 2022-02-17T19:17:59Z | - |
dc.date.issued | 2012 | - |
dc.identifier.issn | 1879-6397 | - |
dc.identifier.uri | http://hdl.handle.net/2445/183269 | - |
dc.description.abstract | Huntington's disease (HD) is an autosomal dominant progressive neurodegenerative disorder due to an expanded CAG/polyglutamine repeat in the coding region of the huntingtin (htt) gene that causes the preferential degeneration of striatal neurons. Although HD is classically considered a motor disorder, cognitive decline manifests even before the appearance of motor symptoms, and reflects the impairment of additional neuronal populations, such as cortical and hippocampal neurons, in the presence of mutant htt (mhtt). Studies on cognitive dysfunction in HD patients have focused on the cortico-striatal pathway. Here we will describe that HD patients and mouse models share many cognitive defects. Alterations in hippocampal synaptic plasticity and function found in HD mouse models highlight that changes in the functioning of the hippocampal formation contribute to cognitive dysfunction in humans. The similarity between the cognitive dysfunction in HD patients and mouse models has helped to understand better how cognitive dysfunction takes place. Moreover, it validates the use of HD mice to study the molecular mechanisms involved in HD cognitive decline. Several studies in HD mouse models indicate that altered synaptic composition/function, deficient neurotrophic support, kinase/phosphatase imbalance, and transcription dysregulation play an important role in cognitive impairment. This knowledge opens the possibility of identifying relevant therapeutic targets to fight cognitive decline in HD. The finding that in HD many mechanisms are similarly altered in hippocampal and striatal neurons suggests the possibility of a common therapeutic strategy to ameliorate both cognitive and motor dysfunction. | - |
dc.format.extent | 19 p. | - |
dc.format.mimetype | application/pdf | - |
dc.language.iso | eng | - |
dc.publisher | IOS Press | - |
dc.relation.isformatof | Reproducció del document publicat a: https://doi.org/10.3233/JHD-120023 | - |
dc.relation.ispartof | Journal of Huntington's disease, 2012, vol. 1, p. 155-173 | - |
dc.relation.uri | https://doi.org/10.3233/JHD-120023 | - |
dc.rights | (c) Giralt Torroella, Albert et al., 2012 | - |
dc.source | Articles publicats en revistes (Biomedicina) | - |
dc.subject.classification | Corea de Huntington | - |
dc.subject.classification | Models animals en la investigació | - |
dc.subject.other | Huntington's chorea | - |
dc.subject.other | Animal models in research | - |
dc.title | Cognitive dysfunction in Huntington¿s disease: Humans, mouse models and molecular mechanisms | - |
dc.type | info:eu-repo/semantics/article | - |
dc.type | info:eu-repo/semantics/publishedVersion | - |
dc.identifier.idgrec | 624227 | - |
dc.date.updated | 2022-02-17T19:17:59Z | - |
dc.rights.accessRights | info:eu-repo/semantics/openAccess | - |
Appears in Collections: | Articles publicats en revistes (IDIBAPS: Institut d'investigacions Biomèdiques August Pi i Sunyer) Articles publicats en revistes (Biomedicina) |
Files in This Item:
File | Description | Size | Format | |
---|---|---|---|---|
624227.pdf | 230.52 kB | Adobe PDF | View/Open |
Items in DSpace are protected by copyright, with all rights reserved, unless otherwise indicated.