Huntington's Disease Research Today is a free monthly online journal that collates and summarizes the latest research about Huntington's Disease, including details on genetics, causes, symptoms, treatment.

The ubiquitin-proteasome system in Huntington's disease.

Valera AG, Díaz-Hernández M, Hernández F, Ortega Z, Lucas JJ

Centro de Biologia Molecular Severo Ochoa, CSIC/UAM, Campus UAM, Cantoblanco, Madrid, Spain.

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.

Published 11 November 2005 in Neuroscientist, 11(6): 583-94.
Full-text of this article is available online (may require subscription).

© 2005-2008 Huntington's Disease Research Today. All Rights Reserved.