Beal M Flint
Department of Neurology and Neuroscience, Weill Medical College of Cornell University, New York Presbyterian Hospital, NY 10021, USA.
Free Radic Res. 2002 Apr;36(4):455-60. doi: 10.1080/10715760290021315.
Coenzyme Q10 (CoQ10) is an essential cofactor of the electron transport gene as well as an important antioxidant, which is particularly effective within mitochondria. A number of prior studies have shown that it can exert efficacy in treating patients with known mitochondrial disorders. We investigated the potential usefulness of coenzyme Q10 in animal models of Parkinson's disease (PD), amyotrophic lateral sclerosis (ALS) and Huntington's disease (HD). It has been demonstrated that CoQ10 can protect against striatal lesions produced by the mitochondrial toxins malonate and 3-nitropropionic acid. These toxins have been utilized to model the striatal pathology, which occurs in HD. It also protects against 1-methyl-1,2,3,6-tetrahydropyridine (MPTP) toxicity in mice. CoQ10 significantly extended survival in a transgenic mouse model of ALS. CoQ10 can significantly extend survival, delay motor deficits and delay weight loss and attenuate the development of striatal atrophy in a transgenic mouse model of HD. In this mouse model, it showed additive efficacy when combined with the N-methyl-D-aspartate (NMDA) receptor antagonist, remacemide. CoQ10 is presently being studied as a potential treatment for early PD as well as in combination with remacemide as a potential treatment for HD.
辅酶Q10（CoQ10）是电子传递基因的必需辅助因子，也是一种重要的抗氧化剂，在线粒体内尤其有效。许多先前的研究表明，它在治疗已知线粒体疾病的患者中可发挥疗效。我们研究了辅酶Q10在帕金森病（PD）、肌萎缩侧索硬化症（ALS）和亨廷顿舞蹈病（HD）动物模型中的潜在效用。已证明CoQ10可保护免受线粒体毒素丙二酸和3-硝基丙酸产生的纹状体损伤。这些毒素已被用于模拟HD中发生的纹状体病理。它还可保护小鼠免受1-甲基-1,2,3,6-四氢吡啶（MPTP）毒性。CoQ10可显著延长ALS转基因小鼠模型的生存期。在HD转基因小鼠模型中，CoQ10可显著延长生存期、延迟运动功能障碍、延迟体重减轻并减轻纹状体萎缩的发展。在该小鼠模型中，当与N-甲基-D-天冬氨酸（NMDA）受体拮抗剂瑞马西胺联合使用时，它显示出相加疗效。目前正在研究CoQ10作为早期PD的潜在治疗方法，以及与瑞马西胺联合作为HD的潜在治疗方法。
