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...

Full description

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

Internet

http://www.sciencedirect.com/science/article/pii/S096999611000183X

Similar Items