title: Therapeutic prospects for the prevention of neurodegeneration in Huntington's disease and the polyglutamine repeat disorders.
abstract: Huntington's 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.
authors.affiliation: Picower Institute for Learning and Memory, Massachusetts Institute of Technology, 46-3243, 43 Vassar St. Cambridge, MA 02139, USA. ykimura@mit.edu
authors.forename: Yoko
authors.initials: Y
authors.lastname: Kimura
authors.forename: Wyan-Ching Mimi
authors.initials: WC
authors.lastname: Lee
authors.forename: J Troy
authors.initials: JT
authors.lastname: Littleton
mesh_terms: D006801:Humans; D006816:Huntington Disease / Q000188:drug therapy* / Q000378:metabolism / Q000473:pathology / Q000517:prevention & control*; D010455:Peptides / Q000378:metabolism*
chemical_list: D010455:Peptides; C097188:polyglutamine