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Publication
Trehalose alleviates the phenotype of Machado–Joseph disease mouse models
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Advisor(s)
Abstract(s)
Machado-Joseph disease (MJD), also known as spinocerebellar ataxia type 3, is the most common of the dominantly inherited ataxias worldwide and is characterized by mutant ataxin-3 aggregation and neuronal degeneration. There is no treatment available to block or delay disease progression. In this work we investigated whether trehalose, a natural occurring disaccharide widely used in food and cosmetic industry, would rescue biochemical, behavioral and neuropathological features of an in vitro and of a severe MJD transgenic mouse model.
Description
Keywords
Machado-Joseph disease Autophagy Spinocerebellar ataxia type 3 Polyglutamine disorder Trehalose
Pedagogical Context
Citation
Publisher
BMC