Beal M F
Neurochemistry Laboratory, Massachusetts General Hospital, Boston 02114, USA.
Biofactors. 1999;9(2-4):261-6. doi: 10.1002/biof.5520090222.
Coenzyme Q10 (CoQ10) is an essential cofactor of the electron transport chain as well as an important antioxidant. Previous studies have suggested that it may exert therapeutic effects in patients with known mitochondrial disorders. We investigated whether it can exert neuroprotective effects in a variety of animal models. We have demonstrated that CoQ10 can protect against striatal lesions produced by both malonate and 3-nitropropionic acid. It also protects against MPTP toxicity in mice. It extended survival in a transgenic mouse model of amyotrophic lateral sclerosis. We demonstrated that oral administration can increase plasma levels in patients with Parkinson's disease. Oral administration of CoQ10 significantly decreased elevated lactate levels in patients with Huntington's disease. These studies therefore raise the prospect that administration of CoQ10 may be useful for the treatment of neurodegenerative diseases.
辅酶Q10（CoQ10）是电子传递链的必需辅助因子，也是一种重要的抗氧化剂。先前的研究表明，它可能对已知线粒体疾病的患者发挥治疗作用。我们研究了它是否能在多种动物模型中发挥神经保护作用。我们已经证明，CoQ10可以保护免受丙二酸和3-硝基丙酸所产生的纹状体损伤。它还能保护小鼠免受MPTP毒性。它延长了肌萎缩侧索硬化转基因小鼠模型的生存期。我们证明口服给药可提高帕金森病患者的血浆水平。口服CoQ10可显著降低亨廷顿病患者升高的乳酸水平。因此，这些研究提出了CoQ10给药可能对神经退行性疾病治疗有用的前景。
