Biochemistry of Mitochondrial Coenzyme Q Biosynthesis

Trends Biochem Sci. 2017 Oct;42(10):824-843. doi: 10.1016/j.tibs.2017.06.008. Epub 2017 Sep 17.


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.

Keywords: CoQ-synthome; biosynthesis; coenzyme Q; complex Q; lipids; metabolon; mitochondria; mitochondrial disease; oxidative phosphorylation; protein complex; ubiquinone.

Publication types

  • Review
  • Research Support, N.I.H., Extramural

MeSH terms

  • Humans
  • Mitochondria / metabolism*
  • Ubiquinone / biosynthesis*


  • Ubiquinone