An experimental model for Huntington's chorea?

Dagmar H. Zeef, Ali Jahanshahi, Rinske Vlamings, Joao Casaca-Carreira, Remco G. Santegoeds, Marcus L. F. Janssen, Mayke Oosterloo, Yasin Temel*

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

5 Citations (Web of Science)


Clinically, Huntington's disease (HD) is well known for the predominant motor symptom chorea, which is a hyperkinetic motor disorder. The only experimental model currently described in the literature, as far as we are aware of, exhibiting hyperkinetic movements is the transgenic rat model of HD. We assessed and characterized these hyperkinetic movements in detail and investigated the effect of tetrabenazine (TBZ) treatment. TBZ is an effective drug in the treatment of chorea in HD patients. Our results showed that the hyperkinetic movements fulfilled the clinical-behavioral criteria of a choreiform movement. Administration of TBZ reduced the number of these hyperkinetic movements substantially. These findings suggest that the hyperkinetic movements observed in this animal model can be considered as a choreiform movement disorder. This makes these animals unique and provides opportunities for chorea-research.
Original languageEnglish
Pages (from-to)31-34
JournalBehavioural Brain Research
Publication statusPublished - 1 Apr 2014


  • Transgenic rat model
  • Huntington's disease
  • Chorea
  • Dopamine
  • Tetrabenazine

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