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

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 Authors:	Anabela Silva-Fernandes, Maria do Carmo Costa, Sara Duarte-Silva, Pedro Oliveira, Claudia M. Botelho, Luís Martins, José António Mariz, Tiago Ferreira, Filipa Ribeiro, Margarida Correia-Neves, Cristina Costa, Patrícia Maciel
Format:	Article
Language:	English
Published:	Elsevier 2010-10-01
Series:	Neurobiology of Disease
Subjects:	Polyglutamine Neuronal atrophy Pathogenesis Neuroinflammation Spinocerebellar ataxia Neurodegeneration
Online Access:	http://www.sciencedirect.com/science/article/pii/S096999611000183X

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