A growing number of neurodegenerative diseases are caused by expansion of CAG trinucleotide repeats coding for polyglutamine. The presence of intranuclear inclusions in the affected neuronal cells has suggested a mechanism for pathogenesis based on protein misfolding and aggregation. Detailed understanding of these phenomena is therefore crucial in order to rationalize different phases of the diseases. In the past decade, a few studies have focused on the structural properties of polyglutamine and on the molecular bases of the aggregation process. Most of these studies have been performed on polyglutamine peptides and protein models. Only one report is currently available on the characterization of a full-length polyglutamine protein. The structural hypotheses resulting from these studies are reviewed here.
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August 2002
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Conference Article|
August 01 2002
Glutamine repeats: structural hypotheses and neurodegeneration
L. Masino;
L. Masino
1National Institute for Medical Research, The Ridgeway, London NW7 1AA, U.K.
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A. Pastore
A. Pastore
1
1National Institute for Medical Research, The Ridgeway, London NW7 1AA, U.K.
1To whom correspondence should be addressed (e-mail apastor@nimr.mrc.ac.uk)
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Publisher: Portland Press Ltd
Received:
March 13 2002
Online ISSN: 1470-8752
Print ISSN: 0300-5127
© 2002 Biochemical Society
2002
Biochem Soc Trans (2002) 30 (4): 548–551.
Article history
Received:
March 13 2002
Citation
L. Masino, A. Pastore; Glutamine repeats: structural hypotheses and neurodegeneration. Biochem Soc Trans 1 August 2002; 30 (4): 548–551. doi: https://doi.org/10.1042/bst0300548
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