Review
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Primary Coenzyme Q10 deficiency: An Update
Version 1
: Received: 8 May 2023 / Approved: 15 May 2023 / Online: 15 May 2023 (10:36:16 CEST)
How to cite: Hargreaves, I.P.; Mantle, D. Primary Coenzyme Q10 deficiency: An Update. Preprints 2023, 2023051024. https://doi.org/10.20944/preprints202305.1024.v1 Hargreaves, I.P.; Mantle, D. Primary Coenzyme Q10 deficiency: An Update. Preprints 2023, 2023051024. https://doi.org/10.20944/preprints202305.1024.v1
Abstract
Abstract
Coenzyme Q10 (CoQ10) has a number of vital functions in all cells, both mitochondrial and extra-mitochondrial. In addition to its key role in mitochondrial oxidative phosphorylation, CoQ10 serves as a lipid soluble antioxidant, plays an important role in fatty acid beta-oxidation and pyrimidine and lysosomal metabolism, as well as directly mediating the expression of a number of genes, including those involved in inflammation. Because of the multiplicity of roles in cell function, it is not surprising that deficiency of CoQ10 has been implicated in the pathogenesis of a wide range of disorders. CoQ10 deficiency is broadly divided into primary and secondary types. Primary CoQ10 deficiency results from mutations in genes involved in the CoQ10 biosynthetic pathway. In man, at least 10 genes are required for the biosynthesis of functional CoQ10, a mutation in any one of which can result in a deficit in CoQ10 status. Patients may respond well to oral CoQ10 supplementation, although the condition must be recognised sufficiently early, before irreversible tissue damage has occurred. In this article, we have reviewed clinical studies (up to March 2023) relating to the identification of these deficiencies, and the therapeutic outcomes of CoQ10 supplementation.
Keywords
coenzyme Q10, oxidative stress, primary deficiency, blood brain barrier, mitochondrial, supplementation
Subject
Biology and Life Sciences, Biochemistry and Molecular Biology
Copyright: This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
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