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.