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Citation
Todd, T.W., Lim, J. (2013). Aggregation formation in the polyglutamine diseases: protection at a cost?  Mol. Cells 36(3): 185--194.
FlyBase ID
FBrf0226161
Publication Type
Review
Abstract

Mutant protein aggregation is a hallmark of many neurodegenerative diseases, including the polyglutamine disorders. Although the correlation between aggregation formation and disease pathology originally suggested that the visible inclusions seen in patient tissue might directly contribute to pathology, additional studies failed to confirm this hypothesis. Current opinion in the field of polyglutamine disease research now favors a model in which large inclusions are cytoprotective and smaller oligomers or misfolded monomers underlie pathogenesis. Nonetheless, therapies aimed at reducing or preventing aggregation show promise. This review outlines the debate about the role of aggregation in the polyglutamine diseases as it has unfolded in the literature and concludes with a brief discussion on the manipulation of aggregation formation and clearance mechanisms as a means of therapeutic intervention.

PubMed ID
PubMed Central ID
PMC3800151 (PMC) (EuropePMC)
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Secondary IDs
    Language of Publication
    English
    Additional Languages of Abstract
    Parent Publication
    Publication Type
    Journal
    Abbreviation
    Mol. Cells
    Title
    Molecules and Cells
    ISBN/ISSN
    1016-8478
    Data From Reference
    Human Disease Models (1)