Disorders of Human Coenzyme Q10 Metabolism: An Overview

Hargreaves, Iain; Heaton, Robert A.; Mantle, David

doi:10.3390/ijms21186695

Cited by 189Coenzyme Q10 studies and effectsMetabolism and Genetic DisordersNeurological diseases and metabolism

Disorders of Human Coenzyme Q10 Metabolism: An Overview

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Hargreaves, Iain, Heaton, Robert A., Mantle, David·2020·International Journal of Molecular Sciences

DOI 10.3390/ijms21186695PMC PMC7555759

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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.

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A lack of CoQ10 has been related to several conditions, including aging and neurological disorders.
“A lack of CoQ10 has been related to several conditions, including aging and neurological disorders [83,84,85,86,87,88,89] (Table 1).”
From The Role of Antioxidants in the Interplay between Oxidative Stress and Senescence (2022) · cites this paper as [83]
CoQ10 is a lipid-soluble molecule involved in oxidative phosphorylation, metabolism, mitochondria permeability and antioxidant defence, either directly or indirectly.
“CoQ10 is a lipid-soluble molecule involved in oxidative phosphorylation, metabolism, mitochondria permeability and antioxidant defence, either directly or indirectly [83].”
From The Role of Antioxidants in the Interplay between Oxidative Stress and Senescence (2022) · cites this paper as [83]

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[16]Salviati L. Trevisson E. Doimo M. Navas P. Primary Coenzyme Q10 Deficiency University of Washington Seattle, WA, USA 2017 28125198

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Authors: Hargreaves, I., Heaton, R. A., & Mantle, D.
Affiliations: Liverpool John Moores University (GB)
Pharma Nord (United Kingdom) (GB)
Corresponding author: Hargreaves, Iain
ORCID: Hargreaves, Iain
Heaton, Robert A.
Venue: International Journal of Molecular Sciences
Publisher: MDPI AG
Published: 2020-09-13
Volume: 21
Issue: 18
Pages: 6695
ISSN: 1422-0067 (ISSN-L)
DOI: 10.3390/ijms21186695
PMC: PMC7555759
OpenAlex: W3085499101
Cited by: 182
References: 93
Citations by year: 2026: 18 · 2025: 44 · 2024: 36 · 2023: 37 · 2022: 35 · 2021: 9 · 2020: 2 · 2017: 1
FWCI: 1.99
Type: journal article
Language: en
Open access: gold
doi.org
Licence: cc-by
creativecommons.org/licenses/by/4.0/ (vor)
Locations: International Journal of Molecular Sciences (publishedVersion, cc-by)
PubMed Central (publishedVersion, cc-by)
PubMed (publishedVersion)
Liverpool John Moores University (submittedVersion)
DOAJ (DOAJ: Directory of Open Access Journals) (submittedVersion, cc-by-sa)
Topics: Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Coenzyme Q10 studies and effects
Life Sciences › Biochemistry, Genetics and Molecular Biology › Clinical Biochemistry › Metabolism and Genetic Disorders
Life Sciences › Neuroscience › Neurology › Neurological diseases and metabolism
Keywords: coenzyme Q10, CoQ10 deficiency, CoQ10 biosynthesis, mitochondrial oxidative phosphorylation, antioxidant function, fatty acid metabolism, lysosomal metabolism, inflammation, coenzyme Q10 supplementation, genetic disorders
MeSH terms: Antioxidants, Ataxia, Humans, Inflammation, Ubiquinone, Muscle Weakness, Mitochondrial Diseases

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Disorders of Human Coenzyme Q10 Metabolism: An Overview

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