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Global changes to the ubiquitin system in Huntington's disease

Author

Listed:
  • Eric J. Bennett

    (Stanford University, Stanford, California 94305, USA)

  • Thomas A. Shaler

    (PPD Biomarker Discovery Inc., 1505 O’Brien Drive, Menlo Park, California 94025, USA)

  • Ben Woodman

    (King’s College London School of Medicine, London SE1 9RT, UK)

  • Kwon-Yul Ryu

    (Stanford University, Stanford, California 94305, USA)

  • Tatiana S. Zaitseva

    (Stanford University, Stanford, California 94305, USA)

  • Christopher H. Becker

    (PPD Biomarker Discovery Inc., 1505 O’Brien Drive, Menlo Park, California 94025, USA)

  • Gillian P. Bates

    (King’s College London School of Medicine, London SE1 9RT, UK)

  • Howard Schulman

    (PPD Biomarker Discovery Inc., 1505 O’Brien Drive, Menlo Park, California 94025, USA)

  • Ron R. Kopito

    (Stanford University, Stanford, California 94305, USA)

Abstract

Ubiquitin inclusions In patients with the neurodegenerative disorder Huntington's disease, the neurons contain abnormal protein inclusion bodies enriched with ubiquitin. It has been suggested that alterations in the cellular ubiquitination and proteasomal degradation system contribute to the disease. A mass spectrometry analysis of the brains of Huntington's disease patients and of several mouse models of the disease confirms that ubiquitin chains are ever-present in protein inclusions, suggesting that a general dysfunction of this system may contribute to the disease process. These observations point to ubiquitin as possible diagnostic tool, and since abnormal enrichment of ubiquitin in neuropathological lesions is also a feature of Alzheimer's disease, it may be that these neurologically diverse diseases share fundamental molecular features.

Suggested Citation

  • Eric J. Bennett & Thomas A. Shaler & Ben Woodman & Kwon-Yul Ryu & Tatiana S. Zaitseva & Christopher H. Becker & Gillian P. Bates & Howard Schulman & Ron R. Kopito, 2007. "Global changes to the ubiquitin system in Huntington's disease," Nature, Nature, vol. 448(7154), pages 704-708, August.
    • Handle: RePEc:nat:nature:v:448:y:2007:i:7154:d:10.1038_nature06022
    DOI: 10.1038/nature06022
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    Cited by:

    1. Nathan Riguet & Anne-Laure Mahul-Mellier & Niran Maharjan & Johannes Burtscher & Marie Croisier & Graham Knott & Janna Hastings & Alice Patin & Veronika Reiterer & Hesso Farhan & Sergey Nasarov & Hila, 2021. "Nuclear and cytoplasmic huntingtin inclusions exhibit distinct biochemical composition, interactome and ultrastructural properties," Nature Communications, Nature, vol. 12(1), pages 1-27, December.

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