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Review
. 2020 Sep 13;21(18):6695.
doi: 10.3390/ijms21186695.

Disorders of Human Coenzyme Q10 Metabolism: An Overview

Iain Hargreaves  1 , Robert A Heaton  1 , David Mantle  2
Affiliations
Review

Disorders of Human Coenzyme Q10 Metabolism: An Overview

Iain Hargreaves et al. Int J Mol Sci. .

Abstract

Coenzyme Q10 (CoQ10) has a number of vital functions in all cells, both mitochondrial and extramitochondrial. In addition to its key role in mitochondrial oxidative phosphorylation, CoQ10 serves as a lipid soluble antioxidant, plays an important role in fatty acid, pyrimidine and lysosomal metabolism, as well as directly mediating the expression of a number of genes, including those involved in inflammation. In view of the central role of CoQ10 in cellular metabolism, it is unsurprising that a CoQ10 deficiency is linked to the pathogenesis of a range of disorders. CoQ10 deficiency is broadly classified into primary or secondary deficiencies. Primary deficiencies result from genetic defects in the multi-step biochemical pathway of CoQ10 synthesis, whereas secondary deficiencies can occur as result of other diseases or certain pharmacotherapies. In this article we have reviewed the clinical consequences of primary and secondary CoQ10 deficiencies, as well as providing some examples of the successful use of CoQ10 supplementation in the treatment of disease.

Keywords: antioxidant; coenzyme Q10; deficiencies; mitochondria; oxidative stress.

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Conflict of interest statement

The authors declare no conflict of interest. The sponsors had no role in the design, execution, interpretation, or writing of the study.

Figures

Figure 1
Figure 1
The chemical structure of CoQ10.
Figure 2
Figure 2
Basic schematic of the mitochondrial electron transport chain (METC), highlighting the electron carrier function of CoQ10 in the chain.
Figure 3
Figure 3
Schematic of the mevalonate pathway responsible for the biosynthesis of CoQ10. Solid arrows represent single biosynthetic reactions; Dashed arrows represent multiple biosynthetic reactions.
Figure 4
Figure 4
Putative mechanisms responsible for secondary CoQ10 in disease in MPS, PKU and METC disorders. Oxidative stress (OS), pyridoxal 5-phosphate (PLP), mucopolysaccharidosis (MPS), phenylketonuria (PKU), phenylalanine (Phe) and mitochondrial electron transport chain (METC). Black arrows represent defined biochemical reactions; red arrows represent tentative biochemical reactions. ‘X‘ represents the inhibition of an enzyme or biosynthetic step.
See this image and copyright information in PMC

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