Choosing an animal model for the study of Huntington's disease

Nat Rev Neurosci. 2013 Oct;14(10):708-21. doi: 10.1038/nrn3570.


Since the identification of the causative gene in Huntington's disease (HD), a number of animal models of this disorder have been developed. A frequently asked question is: which of these models most closely recapitulates the human disease? In this Review, we provide an overview of the currently available animal models of HD in the context of the clinical features of the disease. In doing so, we highlight their strengths and limitations for modelling specific symptoms of the disease. This should highlight the animal model that is best suited to address a particular question of interest and, ultimately, to expedite the discovery of treatments that will prevent or slow the progression of HD.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Animals
  • Animals, Genetically Modified
  • Disease Models, Animal*
  • Humans
  • Huntingtin Protein
  • Huntington Disease* / classification
  • Huntington Disease* / genetics
  • Huntington Disease* / physiopathology
  • Nerve Tissue Proteins / genetics
  • Species Specificity
  • Trinucleotide Repeats / genetics


  • HTT protein, human
  • Huntingtin Protein
  • Nerve Tissue Proteins