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Article

Autophagy induction reduces mutant ataxin-3 levels and toxicity in a mouse model of spinocerebellar ataxia type 3.

Brain : a journal of neurology - 1 Jan 2010

Menzies Fiona M, Huebener Jeannette, Renna Maurizio, Bonin Michael, Riess Olaf, Rubinsztein David C

Abstract excerpt

Spinocerebellar ataxia type 3 is a neurodegenerative disorder caused by the expansion of the polyglutamine repeat region within the ataxin-3 protein. The mutant protein forms intracellular aggregates in the brain. However, the cellular mechanisms causing toxicity are still poorly understood and there are currently no effective treatments. In this study we show that administration of a rapamycin ester (cell cycle...

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Identifiers

PMID: 20007218
DOI: 10.1093/brain/awp292
PMCID: PMC2801325

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