López-Pedrera Chary, Villalba José Manuel, Patiño-Trives Alejandra Mª, Luque-Tévar Maria, Barbarroja Nuria, Aguirre Mª Ángeles, Escudero-Contreras Alejandro, Pérez-Sánchez Carlos
Rheumatology Service, Reina Sofia Hospital/Maimonides Institute for Research in Biomedicine of Córdoba (IMIBIC), University of Córdoba, 14004 Córdoba, Spain.
Department of Cell Biology, Immunology and Physiology, Agrifood Campus of International Excellence, University of Córdoba, ceiA3, 14014 Córdoba, Spain.
Antioxidants (Basel). 2021 Apr 13;10(4):600. doi: 10.3390/antiox10040600.
Coenzyme Q (CoQ) is a mitochondrial electron carrier and a powerful lipophilic antioxidant located in membranes and plasma lipoproteins. CoQ is endogenously synthesized and obtained from the diet, which has raised interest in its therapeutic potential against pathologies related to mitochondrial dysfunction and enhanced oxidative stress. Novel formulations of solubilized CoQ and the stabilization of reduced CoQ (ubiquinol) have improved its bioavailability and efficacy. Synthetic analogues with increased solubility, such as idebenone, or accumulated selectively in mitochondria, such as MitoQ, have also demonstrated promising properties. CoQ has shown beneficial effects in autoimmune diseases. Leukocytes from antiphospholipid syndrome (APS) patients exhibit an oxidative perturbation closely related to the prothrombotic status. In vivo ubiquinol supplementation in APS modulated the overexpression of inflammatory and thrombotic risk-markers. Mitochondrial abnormalities also contribute to immune dysregulation and organ damage in systemic lupus erythematosus (SLE). Idebenone and MitoQ improved clinical and immunological features of lupus-like disease in mice. Clinical trials and experimental models have further demonstrated a therapeutic role for CoQ in Rheumatoid Arthritis, multiple sclerosis and type 1 diabetes. This review summarizes the effects of CoQ and its analogs in modulating processes involved in autoimmune disorders, highlighting the potential of these therapeutic approaches for patients with immune-mediated diseases.
辅酶Q（CoQ）是一种线粒体电子载体，也是一种强大的亲脂性抗氧化剂，存在于细胞膜和血浆脂蛋白中。CoQ可内源性合成，也可从饮食中获取，这引发了人们对其治疗与线粒体功能障碍及氧化应激增强相关疾病的潜力的兴趣。增溶型CoQ的新型制剂以及还原型CoQ（泛醇）的稳定性提高了其生物利用度和疗效。具有更高溶解度的合成类似物，如艾地苯醌，或能选择性地在线粒体中蓄积的类似物，如MitoQ，也显示出了有前景的特性。CoQ在自身免疫性疾病中已显示出有益作用。抗磷脂综合征（APS）患者的白细胞表现出与血栓前状态密切相关的氧化紊乱。对APS患者进行体内泛醇补充可调节炎症和血栓形成风险标志物的过度表达。线粒体异常也会导致系统性红斑狼疮（SLE）的免疫失调和器官损伤。艾地苯醌和MitoQ改善了小鼠狼疮样疾病的临床和免疫学特征。临床试验和实验模型进一步证明了CoQ在类风湿性关节炎、多发性硬化症和1型糖尿病中的治疗作用。本综述总结了CoQ及其类似物在调节自身免疫性疾病相关过程中的作用，强调了这些治疗方法对免疫介导疾病患者的潜力。
