RNA toxicity is a component of ataxin-3 degeneration in Drosophila

Ling-Bo Li, Zhenming Yu, Xiuyin Teng and Nancy M. Bonini ()
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Ling-Bo Li: Department of Biology,
Zhenming Yu: Department of Biology,
Xiuyin Teng: Department of Biology,
Nancy M. Bonini: Department of Biology,

Nature, 2008, vol. 453, issue 7198, 1107-1111

Abstract: Ataxin-3 degeneration: An RNA-mediated component Polyglutamine (polyQ) diseases are a class of dominantly inherited neurodegenerative disorders caused by the expansion of a CAG repeat encoding glutamine within the coding region of the respective genes. PolyQ has traditionally been thought to cause neurodegeneration due to protein toxicity. In contrast, pathogenesis in other repeat diseases, such as myotonic dystrophy and fragile X-associated tremor/ataxia syndrome, is thought to result from the expression of toxic RNA repeats. Here they provide evidence that pathogenesis caused by ataxin-3, which contains CAG repeats, also involves an RNA-mediated component.

Date: 2008
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DOI: 10.1038/nature06909

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