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Disorders of Human Coenzyme Q10 Metabolism: An Overview

Hargreaves, IP, Heaton, RA and Mantle, D (2020) Disorders of Human Coenzyme Q10 Metabolism: An Overview. International Journal of Molecular Sciences, 21 (18). ISSN 1661-6596

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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.

Item Type: Article
Uncontrolled Keywords: 0399 Other Chemical Sciences, 0604 Genetics, 0699 Other Biological Sciences
Subjects: R Medicine > RM Therapeutics. Pharmacology
Divisions: Pharmacy & Biomolecular Sciences
Publisher: MDPI
Date Deposited: 15 Sep 2020 11:23
Last Modified: 04 Sep 2021 06:41
DOI or ID number: 10.3390/ijms21186695
URI: https://researchonline.ljmu.ac.uk/id/eprint/13639
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