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Disorders of Human Coenzyme Q10 Metabolism: An Overview
Open accessAbstract
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 [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]
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“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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Berger, M. M., Shenkin, A., Schweinlin, A., Amrein, K., Augsburger, M., Biesalski, H.-K., Bischoff, S. C., Casaer, M. P., Gundogan, K., Lepp, H.-L., et al. (2022) · Clinical Nutrition · doi:10.1016/j.clnu.2022.02.015 · Cited by 615
Ferroptosis Mechanisms Involved in Neurodegenerative Diseases
Reichert, C. O., de Freitas, F. A., Sampaio‐Silva, J., Rokita-Rosa, L., Barros, P. D. L., Levy, D., & Bydlowski, S. P. (2020) · International Journal of Molecular Sciences · doi:10.3390/ijms21228765 · Cited by 431
Yang, X., Wang, H., Yung, M. M. H., Chen, F., Chan, W.-S., Chan, Y.-S., Tsui, S. K., Ngan, H., Chan, K. K. L., & Chan, D. W. (2022) · Theranostics · doi:10.7150/thno.70194 · Cited by 257
Ferroptosis in Parkinson's disease: Molecular mechanisms and therapeutic potential
Ding, X.-S., Gao, L., Han, Z., Eleuteri, S., Shi, W., Shen, Y., Song, Z.-Y., Su, M., Yang, Q., Qu, Y., et al. (2023) · Ageing Research Reviews · doi:10.1016/j.arr.2023.102077 · Cited by 211
The Role of Antioxidants in the Interplay between Oxidative Stress and Senescence
Varesi, A., Chirumbolo, S., Campagnoli, L. I. M., Pierella, E., Piccini, G. B., Carrara, A., Ricevuti, G., Scassellati, C., Bonvicini, C., & Pascale, A. (2022) · Antioxidants · doi:10.3390/antiox11071224 · Cited by 145
Metadata
- Authors
- Hargreaves, I., Heaton, R. A., & Mantle, D.
- Affiliations
- Liverpool John Moores University (GB)
- Pharma Nord (United Kingdom) (GB)
- Corresponding author
- Hargreaves, Iain
- Venue
- International Journal of Molecular Sciences
- Publisher
- MDPI AG
- Published
- 2020-09-13
- Volume
- 21
- Issue
- 18
- Pages
- 6695
- ISSN
- 1422-0067 (ISSN-L)
- 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
- FWCI
- 1.99
- Type
- journal article
- Language
- en
- Open access
- gold
- doi.org
- Licence
- 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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Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.
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