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Mini-Reviews in Medicinal Chemistry

Editor-in-Chief

ISSN (Print): 1389-5575
ISSN (Online): 1875-5607

Therapeutic Prospects for the Prevention of Neurodegeneration in Huntingtons Disease and the Polyglutamine Repeat Disorders

Author(s): Yoko Kimura, Wyan-Ching Mimi Lee and J. Troy Littleton

Volume 7, Issue 1, 2007

Page: [99 - 106] Pages: 8

DOI: 10.2174/138955707779317867

Price: $65

Abstract

Huntingtons disease (HD) is a dominantly inherited neurodegenerative disorder caused by expansion of a polyglutamine (polyQ) tract in the huntingtin protein, resulting in intracellular aggregate formation and neurodegeneration. Biochemical pathways leading from polyQ expansion to disease pathogenesis are largely unknown. Recent approaches using genetic models systems have begun to uncover nuclear and cytoplasmic pathologies that represent potential targets for therapeutic intervention.

Keywords: Axonal transport, neurodegeneration, vesicle trafficking, protein aggregation, polyQ, huntington's disease


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