Schilling G, Coonfield M L, Ross C A, Borchelt D R
Department of Pathology, The Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA.
Neurosci Lett. 2001 Nov 27;315(3):149-53. doi: 10.1016/s0304-3940(01)02326-6.
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.
亨廷顿舞蹈症（HD）是一种进行性遗传性神经退行性疾病，目前尚无有效治疗方法。最近发表的CARE-HD研究评估了辅酶Q10（CoQ10）和盐酸瑞玛西胺（R）联合使用改善可能由谷氨酸介导的兴奋性毒性和线粒体能量产生异常所引起症状的能力。在本研究中，我们检测了CoQ10/R疗法改善HD-N171-82Q转基因小鼠运动功能障碍和过早死亡的疗效。在开始CoQ10/R治疗3周后，通过转棒试验测量的运动能力有特异性但短暂的改善。然而，生存期并未延长。我们的研究结果表明，有必要在小鼠模型中进一步研究CoQ10/R，以探究这种治疗方法是否能在疾病早期改善HD的症状。
