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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Bibliographic Details
Main Author: Fernandes, Anabela Silva (author)
Other Authors: 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)
Format: article
Language:eng
Published: 2010
Subjects:
Polyglutamine
Neuronal atrophy
Pathogenesis
Neuroinflammation
Spinocerebellar ataxia
Neurodegeneration
Genetic instability
Triplet repeats
Science & Technology
Online Access:http://hdl.handle.net/1822/29574
Country:Portugal
Oai:oai:repositorium.sdum.uminho.pt:1822/29574

Internet

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

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