Abstract
Polyglutamine (polyQ) expansion causes nine inherited neurodegenerative disorders, including Huntingtons Disease, Spinobulbar Muscular Atrophy, Dentatorubral-Pallidoluysian Atrophy, and Spinocerebellar Ataxias 1, 2, 3, 6, 7, and 17. The common pathological feature of these diseases is the formation of intracellular polyglutamine inclusions or aggregates. Previous studies have focused on the intranuclear inclusions and found that polyQ proteins can affect gene transcription. Recent studies have revealed that polyQ proteins and their inclusions in neuronal processes can impair intracellular transport. Impaired intracellular trafficking, particularly in axons, may lead to neuronal dysfunction and early neuropathology. In this review, we will discuss the ways that polyQ proteins affect intracellular trafficking with an emphasis on the events that lead to neurodegeneration in Huntingtons disease.
Current Genomics
Title: Polyglutamine Protein Trafficking and Neurodegeneration
Volume: 6 Issue: 3
Author(s): John R. McGuire, Shi-Hua Li and Xiao-Jiang Li
Affiliation:
Abstract: Polyglutamine (polyQ) expansion causes nine inherited neurodegenerative disorders, including Huntingtons Disease, Spinobulbar Muscular Atrophy, Dentatorubral-Pallidoluysian Atrophy, and Spinocerebellar Ataxias 1, 2, 3, 6, 7, and 17. The common pathological feature of these diseases is the formation of intracellular polyglutamine inclusions or aggregates. Previous studies have focused on the intranuclear inclusions and found that polyQ proteins can affect gene transcription. Recent studies have revealed that polyQ proteins and their inclusions in neuronal processes can impair intracellular transport. Impaired intracellular trafficking, particularly in axons, may lead to neuronal dysfunction and early neuropathology. In this review, we will discuss the ways that polyQ proteins affect intracellular trafficking with an emphasis on the events that lead to neurodegeneration in Huntingtons disease.
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Cite this article as:
McGuire R. John, Li Shi-Hua and Li Xiao-Jiang, Polyglutamine Protein Trafficking and Neurodegeneration, Current Genomics 2005; 6 (3) . https://dx.doi.org/10.2174/1389202053971938
DOI https://dx.doi.org/10.2174/1389202053971938 |
Print ISSN 1389-2029 |
Publisher Name Bentham Science Publisher |
Online ISSN 1875-5488 |
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