TY - JOUR
T1 - An experimental model for Huntington's chorea?
AU - Zeef, Dagmar H.
AU - Jahanshahi, Ali
AU - Vlamings, Rinske
AU - Casaca-Carreira, Joao
AU - Santegoeds, Remco G.
AU - Janssen, Marcus L. F.
AU - Oosterloo, Mayke
AU - Temel, Yasin
PY - 2014/4/1
Y1 - 2014/4/1
N2 - 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.
AB - 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.
KW - Transgenic rat model
KW - Huntington's disease
KW - Chorea
KW - Dopamine
KW - Tetrabenazine
U2 - 10.1016/j.bbr.2013.12.036
DO - 10.1016/j.bbr.2013.12.036
M3 - Article
C2 - 24412685
SN - 0166-4328
VL - 262
SP - 31
EP - 34
JO - Behavioural Brain Research
JF - Behavioural Brain Research
ER -