Coenzyme Q (CoQ, ubiquinone) is a redox-active lipid produced across all domains of life that functions in electron transport and oxidative phosphorylation and whose deficiency causes human diseases. Yet, CoQ biosynthesis has not been fully defined in any organism. Several proteins with unclear molecular functions facilitate CoQ biosynthesis through unknown means, and multiple steps in the pathway are catalyzed by currently unidentified enzymes. Here we highlight recent progress toward filling these knowledge gaps through both traditional biochemistry and cutting-edge ‘omics’ approaches. To help fill the remaining gaps, we present questions framed by the recently discovered CoQ biosynthetic complex and by putative biophysical barriers. Mapping CoQ biosynthesis, metabolism, and transport pathways has great potential to enhance treatment of numerous human diseases.

辅酶Q（辅酶Q，泛醌）是一种在生活的所有领域都具有氧化还原活性的脂质，在电子运输和氧化磷酸化中起作用，其缺乏会导致人类疾病。然而，尚未在任何生物体中完全定义辅酶Q的生物合成。几种分子功能不清楚的蛋白质可通过未知方式促进辅酶Q的生物合成，并且该途径中的多个步骤均由目前未知的酶催化。在这里，我们重点介绍了通过传统生物化学和尖端的“组学”方法在填补这些知识空白方面的最新进展。为了填补剩余的空白，我们提出了一些问题，这些问题是由最近发现的辅酶Q生物合成复合物和推定的生物物理障碍构成的。绘制辅酶Q的生物合成，代谢，