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Disorders of Coenzyme Q10 Metabolism: Causes and Consequences

A special issue of International Journal of Molecular Sciences (ISSN 1422-0067). This special issue belongs to the section "Molecular Endocrinology and Metabolism".

Deadline for manuscript submissions: closed (30 September 2020) | Viewed by 35024

Special Issue Editor

Special Issue Information

Dear Colleagues,

In view of the important roles of coenzyme Q10 (CoQ10) as an electron carrier in the mitochondrial respiratory chain (MRC) and as a lipid soluble antioxidant, a deficit in CoQ10 status would be expected to comprise cellular energy generation, in addition to lowering its antioxidant status. Therefore, not surprisingly, a deficiency in CoQ10 status has been linked to a number of diseases and conditions. Furthermore, the involvement of CoQ10 in gene expression and inflammation may also have important implications for disease pathophysiology. 

The purpose of this Special Issue will be to present a selection of studies and reviews that outline the causes and consequences of primary and secondary CoQ10 deficiencies, the involvement of CoQ10 in disease, the therapeutic use of CoQ10 supplementation, and the appropriateness of determining CoQ10 status for monitoring disease progression and therapeutic intervention.

Dr. Iain P. Hargreaves
Guest Editor

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Published Papers (4 papers)

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Review

13 pages, 955 KiB  
Review
Cellular Consequences of Coenzyme Q10 Deficiency in Neurodegeneration of the Retina and Brain
by Haider Manzar, Dalia Abdulhussein, Timothy E. Yap and M. Francesca Cordeiro
Int. J. Mol. Sci. 2020, 21(23), 9299; https://doi.org/10.3390/ijms21239299 - 6 Dec 2020
Cited by 39 | Viewed by 6963
Abstract
Coenzyme Q10 (CoQ10) is a ubiquitous cofactor in the body, operating in the inner mitochondrial membrane, where it plays a vital role in the generation of adenosine triphosphate (ATP) through the electron transport chain (ETC). In addition to this, CoQ10 serves as an [...] Read more.
Coenzyme Q10 (CoQ10) is a ubiquitous cofactor in the body, operating in the inner mitochondrial membrane, where it plays a vital role in the generation of adenosine triphosphate (ATP) through the electron transport chain (ETC). In addition to this, CoQ10 serves as an antioxidant, protecting the cell from oxidative stress by reactive oxygen species (ROS) as well as maintaining a proton (H+) gradient across lysosome membranes to facilitate the breakdown of cellular waste products. Through the process of ageing, the body becomes deficient in CoQ10, resulting in several systemic manifestations. On a cellular level, one of the consequences of CoQ10 deficiency is apoptosis, which can be visualised in tissues of the central nervous system (CNS). Diseases affecting the retina and brain such as age-related macular degeneration (AMD), glaucoma, Alzheimer’s disease (AD) and Parkinson’s disease (PD) have shown defects in cellular biochemical reactions attributed to reduced levels of CoQ10. Through further research into the pathogenesis of such conditions, the effects of CoQ10 deficiency can be counteracted through supplementation, early detection and intervention. Full article
(This article belongs to the Special Issue Disorders of Coenzyme Q10 Metabolism: Causes and Consequences)
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19 pages, 807 KiB  
Review
Coenzyme Q10 Supplementation for the Reduction of Oxidative Stress: Clinical Implications in the Treatment of Chronic Diseases
by Francisco Miguel Gutierrez-Mariscal, Antonio Pablo Arenas-de Larriva, Laura Limia-Perez, Juan Luis Romero-Cabrera, Elena Maria Yubero-Serrano and Jose López-Miranda
Int. J. Mol. Sci. 2020, 21(21), 7870; https://doi.org/10.3390/ijms21217870 - 23 Oct 2020
Cited by 81 | Viewed by 10657
Abstract
Apart from its main function in the mitochondria as a key element in electron transport, Coenzyme Q10 (CoQ10) has been described as having multiple functions, such as oxidant action in the generation of signals and the control of membrane structure [...] Read more.
Apart from its main function in the mitochondria as a key element in electron transport, Coenzyme Q10 (CoQ10) has been described as having multiple functions, such as oxidant action in the generation of signals and the control of membrane structure and phospholipid and cellular redox status. Among these, the most relevant and most frequently studied function is the potent antioxidant capability of its coexistent redox forms. Different clinical trials have investigated the effect of CoQ10 supplementation and its ability to reduce oxidative stress. In this review, we focused on recent advances in CoQ10 supplementation, its role as an antioxidant, and the clinical implications that this entails in the treatment of chronic diseases, in particular cardiovascular diseases, kidney disease, chronic obstructive pulmonary disease, non-alcoholic fatty liver disease, and neurodegenerative diseases. As an antioxidant, CoQ10 has proved to be of potential use as a treatment in diseases in which oxidative stress is a hallmark, and beneficial effects of CoQ10 have been reported in the treatment of chronic diseases. However, it is crucial to reach a consensus on the optimal dose and the use of different formulations, which vary from ubiquinol or ubiquinone Ubisol-Q10 or Qter®, to new analogues such as MitoQ, before we can draw a clear conclusion about its clinical use. In addition, a major effort must be made to demonstrate its beneficial effects in clinical trials, with a view to making the implementation of CoQ10 possible in clinical practice. Full article
(This article belongs to the Special Issue Disorders of Coenzyme Q10 Metabolism: Causes and Consequences)
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13 pages, 1625 KiB  
Review
Disorders of Human Coenzyme Q10 Metabolism: An Overview
by Iain Hargreaves, Robert A. Heaton and David Mantle
Int. J. Mol. Sci. 2020, 21(18), 6695; https://doi.org/10.3390/ijms21186695 - 13 Sep 2020
Cited by 88 | Viewed by 12166
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 [...] Read more.
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. Full article
(This article belongs to the Special Issue Disorders of Coenzyme Q10 Metabolism: Causes and Consequences)
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14 pages, 1304 KiB  
Review
Coenzyme Q10 Supplementation Improves Adipokine Levels and Alleviates Inflammation and Lipid Peroxidation in Conditions of Metabolic Syndrome: A Meta-Analysis of Randomized Controlled Trials
by Phiwayinkosi V. Dludla, Patrick Orlando, Sonia Silvestri, Fabio Marcheggiani, Ilenia Cirilli, Tawanda M. Nyambuya, Vuyolwethu Mxinwa, Kabelo Mokgalaboni, Bongani B. Nkambule, Rabia Johnson, Sithandiwe E. Mazibuko-Mbeje, Christo J. F. Muller, Johan Louw and Luca Tiano
Int. J. Mol. Sci. 2020, 21(9), 3247; https://doi.org/10.3390/ijms21093247 - 4 May 2020
Cited by 31 | Viewed by 4437
Abstract
Evidence from randomized controlled trials (RCTs) suggests that coenzyme Q10 (CoQ10) can regulate adipokine levels to impact inflammation and oxidative stress in conditions of metabolic syndrome. Here, prominent electronic databases such as MEDLINE, Cochrane Library, and EMBASE were searched for [...] Read more.
Evidence from randomized controlled trials (RCTs) suggests that coenzyme Q10 (CoQ10) can regulate adipokine levels to impact inflammation and oxidative stress in conditions of metabolic syndrome. Here, prominent electronic databases such as MEDLINE, Cochrane Library, and EMBASE were searched for eligible RCTs reporting on any correlation between adipokine levels and modulation of inflammation and oxidative stress in individuals with metabolic syndrome taking CoQ10. The risk of bias was assessed using the modified Black and Downs checklist, while the Grading of Recommendations Assessment, Development and Evaluation (GRADE) tool was used to evaluate the quality of evidence. Results from the current meta-analysis, involving 318 participants, showed that CoQ10 supplementation in individuals with metabolic syndrome increased adiponectin levels when compared to those on placebo (SMD: 1.44 [95% CI: −0.13, 3.00]; I2 = 96%, p < 0.00001). Moreover, CoQ10 supplementation significantly lowered inflammation markers in individuals with metabolic syndrome in comparison to those on placebo (SMD: −0.31 [95% CI: −0.54, −0.08]; I2 = 51%, p = 0.07). Such benefits with CoQ10 supplementation were related to its ameliorative effects on lipid peroxidation by reducing malondialdehyde levels, concomitant to improving glucose control and liver function. The overall findings suggest that optimal regulation of adipokine function is crucial for the beneficial effects of CoQ10 in improving metabolic health. Full article
(This article belongs to the Special Issue Disorders of Coenzyme Q10 Metabolism: Causes and Consequences)
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