Coenzyme Q10 and remacemide hydrochloride ameliorate motor deficits in a Huntington's disease transgenic mouse model

Huntington's disease (HD) is a progressive inherited neurodegenerative diso rder, for which there is no effective therapy. The CARE-HD study, recently published, evaluated the ability of a combination of coenzyme Q10 (CoQ10) a nd remacemide hydrochloride (R) to ameliorate symptoms, which might arise f rom glutamate-mediated excitotoxicity and abnormalities in mitochondrial en ergy production. In this study, we examined the efficacy of CoQ10/R therapy on ameliorating the motor dysfunction and premature death of HD-N171-82Q t ransgenic mice. Motor performance, measured on the Rotarod, was specificall y but transiently improved beginning 3 weeks after initiating the CoQ10/R t herapy. Survival, however was not prolonged. Our findings suggest that furt her study of CoQ10/R in mouse models is warranted to investigate whether th is therapeutic approach can ameliorate the symptoms of HD in early stages o f the disease. (C) 2001 Published by Elsevier Science Ireland Ltd.