The Ubiquitin-Proteasome Pathway in Huntington's Disease

Author:

Mitra Siddhartha12,Finkbeiner Steven123

Affiliation:

1. Gladstone Institute of Neurological Disease, 1650 Owens St., San Francisco, CA 94158, USA

2. Medical Scientist Training Program, University of California, 533 Parnassus Ave, San Francisco, CA 94143, USA

3. Departments of Neurology and Physiology, University of California, 533 Parnassus Ave., San Francisco, CA 94143, USA

Abstract

The accumulation of mutant protein is a common feature of neurodegenerative disease. In Huntington's disease, a polyglutamine expansion in the huntingtin protein triggers neuronal toxicity. Accompanying neuronal death, mutant huntingtin aggregates in large macromolecular structures called inclusion bodies. The function of the machinery for intracellular protein degradation is linked to huntingtin toxicity and components of this machinery colocalize with inclusion bodies. An increasing body of evidence implicates the ubiquitin-proteasome pathway in the failure of cells to degrade mutant huntingtin. A number of potential mechanisms that link compromised ubiquitin-proteasome pathway function and neurodegeneration have been proposed and may offer opportunities for therapeutic intervention.

Funder

National Institutes of Health

Publisher

Hindawi Limited

Subject

General Environmental Science,General Biochemistry, Genetics and Molecular Biology,General Medicine

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