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Polyglutamine Aggregation in Huntington and Related Diseases

  • Saskia Polling
  • Andrew F. Hill
  • Danny M. HattersEmail author
Chapter
Part of the Advances in Experimental Medicine and Biology book series (AEMB)

Abstract

Polyglutamine(polyQ)-expansions in differentproteins cause nine neurodegenerative diseases. While polyQ aggregation is a key pathological hallmark of these diseases, how aggregation relates to pathogenesis remains contentious. In this chapter, we review what is known about the aggregation process and how cells respond and interact with the polyQ-expanded proteins. We cover detailed biophysical and structural studies to uncover the intrinsic features of polyQ aggregates and concomitant effects in the cellular environment. We also examine the functional consequences of polyQ aggregation and how cells may attempt to intervene and guide the aggregation process.

Keywords

Huntington Disease Myotonic Dystrophy Mutant Huntingtin Polyglutamine Disease Tandem Repeat Polymorphism 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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Copyright information

© Landes Bioscience and Springer Science+Business Media 2012

Authors and Affiliations

  • Saskia Polling
    • 1
  • Andrew F. Hill
    • 1
  • Danny M. Hatters
    • 1
    Email author
  1. 1.Department of Biochemistry and Molecular BiologyUniversity of MelbourneMelbourneAustralia

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