Abstract
Polyglutamine (polyQ) diseases are inherited neurodegenerative disorders caused by proteins with expanded polyQ regions. Although the pathological mechanisms of these diseases have not yet been elucidated, the processes of protein misfolding and aggregation seem to be a direct cause of neurodegeneration. Detailed structural information on polyQ proteins is therefore essential in order to understand the mechanisms underlying pathogenesis and to design therapeutic strategies. In the past decade, several studies have investigated the structural properties of polyQ proteins and the molecular basis of aggregation and fibre formation. The results obtained in these studies are reviewed here.
Keywords: polyglutamine, aggregation, neurodegenerative diseases, structure, triplet expansion
Protein & Peptide Letters
Title: Polyglutamine and Neurodegeneration: Structural Aspects
Volume: 11 Issue: 3
Author(s): Laura Masino
Affiliation:
Keywords: polyglutamine, aggregation, neurodegenerative diseases, structure, triplet expansion
Abstract: Polyglutamine (polyQ) diseases are inherited neurodegenerative disorders caused by proteins with expanded polyQ regions. Although the pathological mechanisms of these diseases have not yet been elucidated, the processes of protein misfolding and aggregation seem to be a direct cause of neurodegeneration. Detailed structural information on polyQ proteins is therefore essential in order to understand the mechanisms underlying pathogenesis and to design therapeutic strategies. In the past decade, several studies have investigated the structural properties of polyQ proteins and the molecular basis of aggregation and fibre formation. The results obtained in these studies are reviewed here.
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Cite this article as:
Masino Laura, Polyglutamine and Neurodegeneration: Structural Aspects, Protein & Peptide Letters 2004; 11 (3) . https://dx.doi.org/10.2174/0929866043407147
DOI https://dx.doi.org/10.2174/0929866043407147 |
Print ISSN 0929-8665 |
Publisher Name Bentham Science Publisher |
Online ISSN 1875-5305 |
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