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Article

Striatal and nigral pathology in a lentiviral rat model of Machado-Joseph disease

1 Apr 2008

Abstract excerpt

Machado-Joseph disease (MJD) is a fatal, dominant neurodegenerative disorder. MJD results from polyglutamine repeat expansion in the MJD-1 gene, conferring a toxic gain of function to the ataxin-3 protein. In this study, we aimed at overexpressing ataxin-3 in the rat brain using lentiviral vectors (LV), to generate an in vivo MJD genetic model and, to study the disorder in defined brain regions: substantia nigra,...

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PMID: 18385100
DOI: 10.1093/hmg/ddn106

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Striatal and nigral pathology in a lentiviral rat model of Machado-Joseph diseasePMID 18385100Read ↗

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