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Review

Disorders of Human Coenzyme Q10 Metabolism: An Overview

1
School of Pharmacy, Liverpool John Moores University, L3 5UA Liverpool, UK
2
Pharma Nord (UK) Ltd., Telford Court, Morpeth, NE61 2DB Northumberland, UK
*
Author to whom correspondence should be addressed.
Int. J. Mol. Sci. 2020, 21(18), 6695; https://doi.org/10.3390/ijms21186695
Received: 19 August 2020 / Revised: 8 September 2020 / Accepted: 11 September 2020 / Published: 13 September 2020
(This article belongs to the Special Issue Disorders of Coenzyme Q10 Metabolism: Causes and Consequences)
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. View Full-Text
Keywords: coenzyme Q10; mitochondria; oxidative stress; antioxidant; deficiencies coenzyme Q10; mitochondria; oxidative stress; antioxidant; deficiencies
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MDPI and ACS Style

Hargreaves, I.; Heaton, R.A.; Mantle, D. Disorders of Human Coenzyme Q10 Metabolism: An Overview. Int. J. Mol. Sci. 2020, 21, 6695. https://doi.org/10.3390/ijms21186695

AMA Style

Hargreaves I, Heaton RA, Mantle D. Disorders of Human Coenzyme Q10 Metabolism: An Overview. International Journal of Molecular Sciences. 2020; 21(18):6695. https://doi.org/10.3390/ijms21186695

Chicago/Turabian Style

Hargreaves, Iain, Robert A. Heaton, and David Mantle. 2020. "Disorders of Human Coenzyme Q10 Metabolism: An Overview" International Journal of Molecular Sciences 21, no. 18: 6695. https://doi.org/10.3390/ijms21186695

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