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

[ Vol. 7 , Issue. 1 ]


Yoko Kimura, Wyan-Ching Mimi Lee and J. Troy Littleton   Pages 99 - 106 ( 8 )


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.


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


Picower Institute for Learning and Memory, Massachusetts Institute of Technology, 46-3243, 43 Vassar St.Cambridge, MA 02139, USA.

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