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

Author

Listed:
  • Ling-Bo Li

    (Department of Biology,
    Present address: Department of Biochemistry, University of Utah, Salt Lake City, Utah 84112-5650, USA.)

  • Zhenming Yu

    (Department of Biology,
    Howard Hughes Medical Institute, University of Pennsylvania, Philadelphia, Pennsylvania 19104-6018, USA)

  • Xiuyin Teng

    (Department of Biology,
    Howard Hughes Medical Institute, University of Pennsylvania, Philadelphia, Pennsylvania 19104-6018, USA)

  • Nancy M. Bonini

    (Department of Biology,
    Howard Hughes Medical Institute, University of Pennsylvania, Philadelphia, Pennsylvania 19104-6018, USA)

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.

Suggested Citation

  • Ling-Bo Li & Zhenming Yu & Xiuyin Teng & Nancy M. Bonini, 2008. "RNA toxicity is a component of ataxin-3 degeneration in Drosophila," Nature, Nature, vol. 453(7198), pages 1107-1111, June.
  • Handle: RePEc:nat:nature:v:453:y:2008:i:7198:d:10.1038_nature06909
    DOI: 10.1038/nature06909
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    Cited by:

    1. Brett M. O’Brien & Roumita Moulick & Gabriel Jiménez-Avalos & Nandakumar Rajasekaran & Christian M. Kaiser & Sarah A. Woodson, 2024. "Stick-slip unfolding favors self-association of expanded HTT mRNA," Nature Communications, Nature, vol. 15(1), pages 1-10, December.

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