Institute of Physiology Czech Academy of Sciences, Prague, Czech Republic.
Physiol Res. 2021 Dec 30;70(Suppl4):S683-S714. doi: 10.33549/physiolres.934712.
Coenzyme Q10 (CoQ10), a lipophilic substituted benzoquinone, is present in animal and plant cells. It is endogenously synthetized in every cell and involved in a variety of cellular processes. CoQ10 is an obligatory component of the respiratory chain in inner mitochondrial membrane. In addition, the presence of CoQ10 in all cellular membranes and in blood. It is the only endogenous lipid antioxidant. Moreover, it is an essential factor for uncoupling protein and controls the permeability transition pore in mitochondria. It also participates in extramitochondrial electron transport and controls membrane physicochemical properties. CoQ10 effects on gene expression might affect the overall metabolism. Primary changes in the energetic and antioxidant functions can explain its remedial effects. CoQ10 supplementation is safe and well-tolerated, even at high doses. CoQ10 does not cause any serious adverse effects in humans or experimental animals. New preparations of CoQ10 that are less hydrophobic and structural derivatives, like idebenone and MitoQ, are being developed to increase absorption and tissue distribution. The review aims to summarize clinical and experimental effects of CoQ10 supplementations in some neurological diseases such as migraine, Parkinson´s disease, Huntington´s disease, Alzheimer´s disease, amyotrophic lateral sclerosis, Friedreich´s ataxia or multiple sclerosis. Cardiovascular hypertension was included because of its central mechanisms controlling blood pressure in the brainstem rostral ventrolateral medulla and hypothalamic paraventricular nucleus. In conclusion, it seems reasonable to recommend CoQ10 as adjunct to conventional therapy in some cases. However, sometimes CoQ10 supplementations are more efficient in animal models of diseases than in human patients (e.g. Parkinson´s disease) or rather vague (e.g. Friedreich´s ataxia or amyotrophic lateral sclerosis).
辅酶 Q10（CoQ10），一种亲脂性取代苯醌，存在于动物和植物细胞中。它在每个细胞中都由内源性合成，并参与多种细胞过程。CoQ10 是线粒体内膜呼吸链的必需组成部分。此外，CoQ10 存在于所有细胞膜和血液中。它是唯一的内源性脂质抗氧化剂。此外，它是解偶联蛋白的必需因子，并控制线粒体中的通透性转换孔。它还参与细胞外电子传递并控制膜的物理化学性质。CoQ10 对基因表达的影响可能会影响整体代谢。能量和抗氧化功能的主要变化可以解释其治疗作用。CoQ10 补充剂是安全且耐受良好的，即使在高剂量下也是如此。CoQ10 不会在人类或实验动物中引起任何严重的不良反应。正在开发更疏水性和结构衍生物的 CoQ10 新制剂，如艾地苯醌和 MitoQ，以增加吸收和组织分布。综述旨在总结 CoQ10 补充在一些神经疾病中的临床和实验效果，如偏头痛、帕金森病、亨廷顿病、阿尔茨海默病、肌萎缩侧索硬化症、弗里德里希共济失调或多发性硬化症。包括心血管高血压是因为它控制脑干延髓腹外侧区和下丘脑室旁核血压的中枢机制。总之，在某些情况下，建议将 CoQ10 作为常规治疗的辅助治疗似乎是合理的。然而，有时 CoQ10 补充在疾病的动物模型中比在人类患者中更有效（例如帕金森病），或者更模糊（例如弗里德里希共济失调或肌萎缩侧索硬化症）。
