The Complexity of Making Ubiquinone

Trends Endocrinol Metab. 2019 Dec;30(12):929-943. doi: 10.1016/j.tem.2019.08.009. Epub 2019 Oct 7.


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.

Keywords: ER–mitochondria contact sites; coenzyme Q; mitochondrial dysfunction; ubiquinone; ubiquinone biosynthesis.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Animals
  • Endoplasmic Reticulum / metabolism
  • Humans
  • Mitochondria / metabolism*
  • Mitochondrial Diseases / metabolism*
  • Reactive Oxygen Species / metabolism
  • Ubiquinone / metabolism*


  • Reactive Oxygen Species
  • Ubiquinone