Ubiquinone (UQ, coenzyme Q) is an essential electron transfer lipid in the mitochondrial respiratory chain. It is a main source of mitochondrial reactive oxygen species (ROS) but also has antioxidant properties. This mix of characteristics is why ubiquinone supplementation is considered a potential therapy for many diseases involving mitochondrial dysfunction. Mutations in the ubiquinone biosynthetic pathway are increasingly being identified in patients. Furthermore, secondary ubiquinone deficiency is a common finding associated with mitochondrial disorders and might exacerbate these conditions. Recent developments have suggested that ubiquinone biosynthesis occurs in discrete domains of the mitochondrial inner membrane close to ER-mitochondria contact sites. This spatial requirement for ubiquinone biosynthesis could be the link between secondary ubiquinone deficiency and mitochondrial dysfunction, which commonly results in loss of mitochondrial structural integrity.

中文翻译：

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制造泛醌的复杂性

泛醌（UQ，辅酶Q）是线粒体呼吸链中必不可少的电子传递脂质。它是线粒体活性氧 (ROS) 的主要来源，但也具有抗氧化特性。这种混合特征就是为什么补充泛醌被认为是许多涉及线粒体功能障碍的疾病的潜在疗法。越来越多的患者发现泛醌生物合成途径中的突变。此外，继发性泛醌缺乏是与线粒体疾病相关的常见发现，可能会加剧这些疾病。最近的发展表明泛醌生物合成发生在靠近 ER-线粒体接触位点的线粒体内膜的离散域中。