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Mitochondrial dysfunction and unfolded protein response in the pathogenesis of SCA3 in primary cell culture experiments

C Prell, J Hübener, G Krebiehl, R Krüger, F Gellerich, O Riess · Aktuelle Neurologie · Ausgabe 10/2009

DOI 10.1055/s-0029-1238857

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Autor:innenC Prell, J Hübener, G Krebiehl, R Krüger, F Gellerich, O Riess
ZeitschriftAktuelle Neurologie
Ausgabe10/2009
Jahrgang / SeitenNicht angegeben

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Abstract-Vorschau

The spinocerebellar ataxia type 3 (SCA3), also known as Machado-Joseph disease (MJD), is an autosomal dominantly inherited neurodegenerative disorder caused by an expanded CAG repeat in the ataxin-3 gene. To consider the correlation between mitochondrial dysfunction and the pathogenesis of SCA3, we analysed mouse embryonic fibroblasts (MEF), which derived from SCA3 mutant mice, generated by the gene trap (gt) approa…

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C Prell, J Hübener, G Krebiehl, R Krüger, F Gellerich, O Riess (2009) Mitochondrial dysfunction and unfolded protein response in the pathogenesis of SCA3 in primary cell culture experiments. Aktuelle Neurologie (10). https://doi.org/10.1055/s-0029-1238857

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