Motor uncoordination and neuropathology in a transgenic mouse model of Machado-Joseph disease lacking intranuclear inclusions and ataxin-3 cleavage products

Machado-Joseph disease (MJD) is a late-onset neurodegenerative disorder caused by a polyglutamine (polyQ) expansion in the ataxin-3 protein. We generated two transgenic mouse lineages expressing the expanded human ataxin-3 under the control of the CMV promoter: CMVMJD83 and CMVMJD94, carrying Q83 an...

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Detalhes bibliográficos
Autor principal: Fernandes, Anabela Silva (author)
Outros Autores: Costa, Maria do Carmo (author), Silva, Sara Carina Duarte (author), Oliveira, Pedro (author), Botelho, C. M. (author), Martins, Luís Filipe Forte Oliveira (author), Mariz, José (author), Ferreira, Tiago (author), Ribeiro, Filipa Pinto (author), Neves, Margarida Correia (author), Maciel, P. (author), Costa, Cristina (author)
Formato: article
Idioma:eng
Publicado em: 2010
Assuntos:
Polyglutamine
Neuronal atrophy
Pathogenesis
Neuroinflammation
Spinocerebellar ataxia
Neurodegeneration
Genetic instability
Triplet repeats
Science & Technology
Texto completo:http://hdl.handle.net/1822/29574
País:Portugal
Oai:oai:repositorium.sdum.uminho.pt:1822/29574

Texto Completo

http://hdl.handle.net/1822/29574

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