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Molecular Neurodegeneration
Published in: Molecular Neurodegeneration 1/2006

Open Access 01-12-2006 | Review

Multiple pathways contribute to the pathogenesis of Huntington disease

Authors: Shihua Li, Xiao-Jiang Li

Published in: Molecular Neurodegeneration | Issue 1/2006

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Abstract

Huntington disease (HD) is caused by expansion of a polyglutamine (polyQ) domain in the protein known as huntingtin (htt), and the disease is characterized by selective neurodegeneration. Expansion of the polyQ domain is not exclusive to HD, but occurs in eight other inherited neurodegenerative disorders that show distinct neuropathology. Yet in spite of the clear genetic defects and associated neurodegeneration seen with all the polyQ diseases, their pathogenesis remains elusive. The present review focuses on HD, outlining the effects of mutant htt in the nucleus and neuronal processes as well as the role of cell-cell interactions in HD pathology. The widespread expression and localization of mutant htt and its interactions with a variety of proteins suggest that mutant htt engages multiple pathogenic pathways. Understanding these pathways will help us to elucidate the pathogenesis of HD and to target therapies effectively.
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Metadata
Title
Multiple pathways contribute to the pathogenesis of Huntington disease
Authors
Shihua Li
Xiao-Jiang Li
Publication date
01-12-2006
Publisher
BioMed Central
Published in
Molecular Neurodegeneration / Issue 1/2006
Electronic ISSN: 1750-1326
DOI
https://doi.org/10.1186/1750-1326-1-19

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