Animal models of Huntington's disease

Shilpa Ramaswamy, Jodi L. McBride, Jeffrey H. Kordower

    Research output: Contribution to journalReview article

    139 Scopus citations

    Abstract

    Huntington's disease (HD) is a neurological disorder caused by a genetic mutation in the IT15 gene. Progressive cell death in the striatum and cortex, and accompanying declines in cognitive, motor, and psychiatric functions, are characteristic of the disease. Animal models of HD have provided insight into disease pathology and the outcomes of therapeutic strategies. Earlier studies of HD most often used toxin-induced models to study mitochondrial impairment and excitotoxicity-induced cell death, which are both mechanisms of degeneration seen in the HD brain. These models, based on 3-nitropropionic acid and quinolinic acid, respectively, are still often used in HD studies. The discovery in 1993 of the huntingtin mutation led to the creation of newer models that incorporate a similar genetic defect. These models, which include transgenic and knock-in rodents, are more representative of the HD progression and pathology. An even more recent model that uses a viral vector to encode the gene mutation in specific areas of the brain may be useful in nonhuman primates, as it is difficult to produce genetic models in these species. This article examines the aforementioned models and describes their use in HD research, including aspects of the creation, delivery, pathology, and tested therapies for each model.

    Original languageEnglish (US)
    Pages (from-to)356-373
    Number of pages18
    JournalILAR Journal
    Volume48
    Issue number4
    DOIs
    StatePublished - Jan 1 2007

    Keywords

    • 3-Nitropropionic acid
    • Animal models
    • Huntington's disease
    • Knock-in mice
    • Quinolinic acid
    • Transgenic rodents

    ASJC Scopus subject areas

    • Animal Science and Zoology
    • Biochemistry, Genetics and Molecular Biology(all)

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