The Ubiquitin-Proteasome System in Huntington’s Disease-Reference-Cited by-同舟云学术

The Ubiquitin-Proteasome System in Huntington’s Disease

Published:2005-12 Issue:6 Volume:11 Page:583-594
ISSN:1073-8584
Container-title:The Neuroscientist
language:en
Short-container-title:Neuroscientist

Author:

Valera A. G.,Díaz-Hernández M.,HernÁNdez F.,Ortega Z.,Lucas J. J.¹

Affiliation:

1. 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, Spain

Abstract

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.

Publisher

SAGE Publications

Subject

Clinical Neurology,General Neuroscience

Link

http://journals.sagepub.com/doi/pdf/10.1177/1073858405280639

Reference53 articles.

1. Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death

2. Impairment of the Ubiquitin-Proteasome System by Protein Aggregation

3. Global Impairment of the Ubiquitin-Proteasome System by Nuclear or Cytoplasmic Protein Aggregates Precedes Inclusion Body Formation

4. Molecular pathways to neurodegeneration

5. Neuronal dysfunction in a polyglutamine disease model occurs in the absence of ubiquitin–proteasome system impairment and inversely correlates with the degree of nuclear inclusion formation

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