Animal models of Huntington's disease

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

Research output: Contribution to journalReview articlepeer-review

183 Scopus citations


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
Issue number4
StatePublished - 2007
Externally publishedYes


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

ASJC Scopus subject areas

  • General Medicine


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