TY - JOUR
T1 - Coenzyme Q10 and remacemide hydrochloride ameliorate motor deficits in a Huntington's disease transgenic mouse model
AU - Schilling, Gabriele
AU - Coonfield, Michael L.
AU - Ross, Christopher A.
AU - Borchelt, David R.
N1 - Funding Information:
This work has been supported by grants from the Hereditary Disease Foundation and the Huntington's Disease Society of America. Remacemide hydrochloride was generously donated by Astrazeneca, while coenzyme Q10 was supplied from Vitaline Formulas.
PY - 2001/11/27
Y1 - 2001/11/27
N2 - Huntington's disease (HD) is a progressive inherited neurodegenerative disorder, for which there is no effective therapy. The CARE-HD study, recently published, evaluated the ability of a combination of coenzyme Q10 (CoQ10) and remacemide hydrochloride (R) to ameliorate symptoms, which might arise from glutamate-mediated excitotoxicity and abnormalities in mitochondrial energy production. In this study, we examined the efficacy of CoQ10/R therapy on ameliorating the motor dysfunction and premature death of HD-N171-82Q transgenic mice. Motor performance, measured on the Rotarod, was specifically but transiently improved beginning 3 weeks after initiating the CoQ10/R therapy. Survival, however was not prolonged. Our findings suggest that further study of CoQ10/R in mouse models is warranted to investigate whether this therapeutic approach can ameliorate the symptoms of HD in early stages of the disease.
AB - Huntington's disease (HD) is a progressive inherited neurodegenerative disorder, for which there is no effective therapy. The CARE-HD study, recently published, evaluated the ability of a combination of coenzyme Q10 (CoQ10) and remacemide hydrochloride (R) to ameliorate symptoms, which might arise from glutamate-mediated excitotoxicity and abnormalities in mitochondrial energy production. In this study, we examined the efficacy of CoQ10/R therapy on ameliorating the motor dysfunction and premature death of HD-N171-82Q transgenic mice. Motor performance, measured on the Rotarod, was specifically but transiently improved beginning 3 weeks after initiating the CoQ10/R therapy. Survival, however was not prolonged. Our findings suggest that further study of CoQ10/R in mouse models is warranted to investigate whether this therapeutic approach can ameliorate the symptoms of HD in early stages of the disease.
KW - Coenzyme Q10
KW - Drug trial
KW - HD-N171-82Q transgenic mice
KW - Huntington's disease
KW - Intranuclear inclusions
KW - Remacemide hydrochloride
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U2 - 10.1016/S0304-3940(01)02326-6
DO - 10.1016/S0304-3940(01)02326-6
M3 - Article
C2 - 11716985
AN - SCOPUS:0035960544
SN - 0304-3940
VL - 315
SP - 149
EP - 153
JO - Neuroscience Letters
JF - Neuroscience Letters
IS - 3
ER -