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The Neuroscientist, Vol. 11, No. 6, 583-594 (2005)
DOI: 10.1177/1073858405280639

Reviews

The Ubiquitin-Proteasome System in Huntington’s Disease

A. G. Valera

M. Díaz-Hernández

F. HernÁNdez

Z. Ortega

J. J. Lucas

Centro de Biologia Molecular "Severo Ochoa," CSIC/UAM, Campus UAM, Cantoblanco, Madrid, Spain Centro de Biologia Molecular Severo Ochoa. Facultad de Ciencias, Campus UAM de Cantoblanco, 28049, Madrid, Spainjjlucas{at}cbm.uam.es

The main histopathological feature of Huntington’s disease (HD) is the presence of protein aggregates that are gathered into inclusion bodies. So far the mechanisms that lead to inclusion formation as well as their role in the pathogenesis of HD are not totally understood. However, it is well established that inclusion bodies contain components of the ubiquitin-proteasome system. Accordingly, it has been postulated that impairment of this machinery can be one of the causes of this disorder. In this review, the authors summarize the state of current knowledge about this hypothesis.

Key Words: PolyQ diseases • Proteasome • Inclusion body • Aggregate proteins


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