Skip to main content

Thank you for visiting nature.com. You are using a browser version with limited support for CSS. To obtain the best experience, we recommend you use a more up to date browser (or turn off compatibility mode in Internet Explorer). In the meantime, to ensure continued support, we are displaying the site without styles and JavaScript.

  • News & Views
  • Published:

Huntington's disease

In reverse gear

Nature volume 404, pages 944–945 (2000)Cite this article

Yamamoto and colleagues1, writing in Cell, describe a mouse model of Huntington's disease with a difference: the pathological features and symptoms of the disease can be reversed. These findings are both dramatic and unexpected, and indicate — with the obvious caveat that we do not yet know how these results will translate to humans — that it may be possible to treat this devastating disease after symptoms appear.

Huntington's disease is an inherited, late-onset neurodegenerative disease for which there is no known cure. In the Western world it affects about 1 in 10,000 people, with a slightly larger number than this being at risk of developing the disease. Symptoms include changes in personality, cognitive decline and a movement disorder (such as uncontrollable, jerky movements, called chorea)2.

This is a preview of subscription content, access via your institution

Access options

Buy this article

  • Purchase on SpringerLink
  • Instant access to the full article PDF.

USD 39.95

Prices may be subject to local taxes which are calculated during checkout

Figure 1: Progression and reversal of Huntington's disease in a mouse model.

References

  1. Yamamoto, A., Lucas, J. J. & Hen, R. Cell 101, 57–66 (2000).

    Article  CAS  Google Scholar 

  2. Harper, P. S. Huntington's Disease (Saunders, London, 1996).

    Google Scholar 

  3. Huntington's Disease Collaborative Research Group Cell 72, 971–983 ( 1993).

  4. Vonsattel, J.-P et al. J. Neuropathol. Exp. Neurol. 44, 559 –577 (1985).

    Article  CAS  Google Scholar 

  5. DiFiglia, M. et al. Science 277, 1990– 1993 (1997).

    Article  CAS  Google Scholar 

  6. Gutekunst, C.-A et al. J. Neurosci. 19, 2522– 2534 (1999).

    Article  CAS  Google Scholar 

  7. Mangiarini, L. et al. Cell 87, 493–506 (1996).

    Article  CAS  Google Scholar 

  8. Davies, S. W. et al. Cell 90, 537–548 (1997).

    Article  CAS  Google Scholar 

  9. Li, H. et al. Hum. Mol. Genet. 8, 1227– 1236 (1999).

    Article  CAS  Google Scholar 

  10. Cha, J.-H et al. Phil. Trans. R. Soc. Lond. B 354, 981– 989 (1999).

    Article  CAS  Google Scholar 

  11. Augood, S. J., Faull, R. L. & Emson P. C. Ann. Neurol. 42, 215– 221 (1997).

    Article  CAS  Google Scholar 

  12. Gossen, M. & Bujard, H. Proc. Natl Acad. Sci. USA 89, 5547–5551 (1992).

    Article  ADS  CAS  Google Scholar 

  13. Scherzinger, E. et al. Cell 90, 549–558 (1997).

    Article  CAS  Google Scholar 

  14. Kasantsev, A. et al. Proc. Natl Acad. Sci. USA 96, 11404 –11409 (1999).

    Article  ADS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Division of Medical and Molecular Genetics, GKT School of Medicine, King's College, 8th Floor Guy's Tower, Guy's Hospital, London, SE1 9RT, UK

    Gillian Bates

Authors
  1. Gillian Bates
    View author publications

    Search author on:PubMed Google Scholar

Corresponding author

Correspondence to Gillian Bates.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Bates, G. In reverse gear. Nature 404, 944–945 (2000). https://doi.org/10.1038/35010254

Download citation

  • Issue date:

  • DOI: https://doi.org/10.1038/35010254

Search

Advanced search

Quick links

Nature Briefing

Sign up for the Nature Briefing newsletter — what matters in science, free to your inbox daily.

Get the most important science stories of the day, free in your inbox. Sign up for Nature Briefing