The Ubiquitous and Multifaceted Coenzyme Q

A special issue of Antioxidants (ISSN 2076-3921).

Deadline for manuscript submissions: closed (30 September 2023) | Viewed by 17858

Special Issue Editors


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Guest Editor
Centro Andaluz de Biología del Desarrollo, Instituto de Salud Carlos III, Universidad Pablo de Olavide-CSIC-JA, and CIBERER, 41013 Sevilla, Spain
Interests: mitochondria; mitochondria diseases; mitochondria aging; coenzyme Q biosynthesis; coenzyme Q10; aging in mice; dietary fat; coenzime Q deficiency
Department of Life and Environmental Sciences (DISVA), Marche Polytechnic University, Ancona, Italy
Interests: oxidative stress, ageing, mitochondrial dysfunction, bioactive quinones, Coenzyme Q10
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Special Issue Information

Dear Colleagues,

Welcome to the 10th Conference of the International Coenzyme Q10 Association to be held under a hybrid format at Beiersdorf Auditorium, Hamburg, from 12 to 15 May 2022 (https://icqaproject.org/). We are looking forward to seeing you at this event. This Special Issue will publish selected papers based on the new discoveries presented during the meeting, and will include the insights and perspectives on:

  • Coenzyme Q in bioenergetics, including electron transport chain structure as well as mitochondrial ROS damage and signaling.
  • Biosynthesis and its defects in disease onset and progression.
  • Neurological effects of disrupting CoQ homeostasis. 
  • Skin response to CoQ supplementation.
  • Prooxidant vs. antioxidant functions of coenzyme Q.
  • Mitochondrial and non-mitochondrial CoQ in health- and lifespan.

We expect that this conference will present new and exciting developments on the role of coenzyme Q in health and disease, as well as new insights into its biosynthesis pathway. The Scientific Committee cordially welcomes you, and we look forward to your contribution.

Prof. Dr. Plácido Navas
Dr. Luca Tiano
Guest Editors

Manuscript Submission Information

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Keywords

  • Coenzyme Q
  • CoQ homeostasis
  • CoQ supplementation
  • Mitochondrial
  • ROS

Published Papers (6 papers)

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Research

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17 pages, 4094 KiB  
Article
An AlphaFold Structure Analysis of COQ2 as Key a Component of the Coenzyme Q Synthesis Complex
by María de los Ángeles Vargas-Pérez, Damien Paul Devos and Guillermo López-Lluch
Antioxidants 2024, 13(4), 496; https://doi.org/10.3390/antiox13040496 - 21 Apr 2024
Viewed by 1540
Abstract
Coenzyme Q (CoQ) is a lipidic compound that is widely distributed in nature, with crucial functions in metabolism, protection against oxidative damage and ferroptosis and other processes. CoQ biosynthesis is a conserved and complex pathway involving several proteins. COQ2 is a member of [...] Read more.
Coenzyme Q (CoQ) is a lipidic compound that is widely distributed in nature, with crucial functions in metabolism, protection against oxidative damage and ferroptosis and other processes. CoQ biosynthesis is a conserved and complex pathway involving several proteins. COQ2 is a member of the UbiA family of transmembrane prenyltransferases that catalyzes the condensation of the head and tail precursors of CoQ, which is a key step in the process, because its product is the first intermediate that will be modified in the head by the next components of the synthesis process. Mutations in this protein have been linked to primary CoQ deficiency in humans, a rare disease predominantly affecting organs with a high energy demand. The reaction catalyzed by COQ2 and its mechanism are still unknown. Here, we aimed at clarifying the COQ2 reaction by exploring possible substrate binding sites using a strategy based on homology, comprising the identification of available ligand-bound homologs with solved structures in the Protein Data Bank (PDB) and their subsequent structural superposition in the AlphaFold predicted model for COQ2. The results highlight some residues located on the central cavity or the matrix loops that may be involved in substrate interaction, some of which are mutated in primary CoQ deficiency patients. Furthermore, we analyze the structural modifications introduced by the pathogenic mutations found in humans. These findings shed new light on the understanding of COQ2’s function and, thus, CoQ’s biosynthesis and the pathogenicity of primary CoQ deficiency. Full article
(This article belongs to the Special Issue The Ubiquitous and Multifaceted Coenzyme Q)
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13 pages, 1212 KiB  
Article
CoQ10Phytosomes Improve Cellular Ubiquinone Uptake in Skeletal Muscle Cells: An Ex Vivo Study Using CoQ10-Enriched Low-Density Lipoproteins Obtained in a Randomized Crossover Study
by Fabio Marcheggiani, Patrick Orlando, Sonia Silvestri, Ilenia Cirilli, Antonella Riva, Giovanna Petrangolini, Francesca Orsini and Luca Tiano
Antioxidants 2023, 12(4), 964; https://doi.org/10.3390/antiox12040964 - 20 Apr 2023
Viewed by 1960
Abstract
Coenzyme Q10 (CoQ10) bioavailability in vivo is limited due to its lipophilic nature. Moreover, a large body of evidence in the literature shows that muscle CoQ10 uptake is limited. In order to address cell specific differences in CoQ uptake, [...] Read more.
Coenzyme Q10 (CoQ10) bioavailability in vivo is limited due to its lipophilic nature. Moreover, a large body of evidence in the literature shows that muscle CoQ10 uptake is limited. In order to address cell specific differences in CoQ uptake, we compared cellular CoQ10 content in cultured human dermal fibroblasts and murine skeletal muscle cells that were incubated with lipoproteins from healthy volunteers and enriched with different formulations of CoQ10 following oral supplementation. Using a crossover design, eight volunteers were randomized to supplement 100 mg/daily CoQ10 for two weeks, delivered both in phytosome form (UBQ) as a lecithin formulation and in CoQ10 crystalline form. After supplementation, plasma was collected for CoQ10 determination. In the same samples, low density lipoproteins (LDL) were extracted and normalized for CoQ10 content, and 0.5 µg/mL in the medium were incubated with the two cell lines for 24 h. The results show that while both formulations were substantially equivalent in terms of plasma bioavailability in vivo, UBQ-enriched lipoproteins showed a higher bioavailability compared with crystalline CoQ10-enriched ones both in human dermal fibroblasts (+103%) and in murine skeletal myoblasts (+48%). Our data suggest that phytosome carriers might provide a specific advantage in delivering CoQ10 to skin and muscle tissues. Full article
(This article belongs to the Special Issue The Ubiquitous and Multifaceted Coenzyme Q)
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17 pages, 3327 KiB  
Article
CoQ Regulates Brown Adipose Tissue Respiration and Uncoupling Protein 1 Expression
by Ching-Fang Chang, Amanda L. Gunawan, Irene Liparulo, Peter-James H. Zushin, Ambre M. Bertholet, Yuriy Kirichok and Andreas Stahl
Antioxidants 2023, 12(1), 14; https://doi.org/10.3390/antiox12010014 - 22 Dec 2022
Cited by 4 | Viewed by 2440
Abstract
Coenzyme Q (CoQ, aka ubiquinone) is a key component of the mitochondrial electron transport chain (ETC) and membrane-incorporated antioxidant. CoQ10 deficiencies encompass a heterogeneous spectrum of clinical phenotypes and can be caused by hereditary mutations in the biosynthesis pathway or result from pharmacological [...] Read more.
Coenzyme Q (CoQ, aka ubiquinone) is a key component of the mitochondrial electron transport chain (ETC) and membrane-incorporated antioxidant. CoQ10 deficiencies encompass a heterogeneous spectrum of clinical phenotypes and can be caused by hereditary mutations in the biosynthesis pathway or result from pharmacological interventions such as HMG-CoA Reductase inhibitors, and statins, which are widely used to treat hypercholesterolemia and prevent cardiovascular disease. How CoQ deficiency affects individual tissues and cell types, particularly mitochondrial-rich ones such as brown adipose tissue (BAT), has remained poorly understood. Here we show that pharmacological and genetic models of BAT CoQ deficiency show altered respiration that can only in part be explained by classical roles of CoQ in the respiration chain. Instead, we found that CoQ strongly impacts brown and beige adipocyte respiration via the regulation of uncoupling protein 1 (UCP1) expression. CoQ deficiency in BAT robustly decreases UCP1 protein levels and uncoupled respiration unexpectedly, resulting in increased inner mitochondrial membrane potential and decreased ADP/ATP ratios. Suppressed UCP1 expression was also observed in a BAT-specific in vivo model of CoQ deficiency and resulted in enhanced cold sensitivity. These findings demonstrate an as yet unappreciated role of CoQ in the transcriptional regulation of key thermogenic genes and functions. Full article
(This article belongs to the Special Issue The Ubiquitous and Multifaceted Coenzyme Q)
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14 pages, 2577 KiB  
Article
Assessing Cellular Uptake of Exogenous Coenzyme Q10 into Human Skin Cells by X-ray Fluorescence Imaging
by Theresa Staufer, Mirja L. Schulze, Oliver Schmutzler, Christian Körnig, Vivienne Welge, Thorsten Burkhardt, Jens-Peter Vietzke, Alexandra Vogelsang, Julia M. Weise, Thomas Blatt, Oliver Dabrowski, Gerald Falkenberg, Dennis Brückner, Carlos Sanchez-Cano and Florian Grüner
Antioxidants 2022, 11(8), 1532; https://doi.org/10.3390/antiox11081532 - 6 Aug 2022
Cited by 2 | Viewed by 3474
Abstract
X-ray fluorescence (XRF) imaging is a highly sensitive non-invasive imaging method for detection of small element quantities in objects, from human-sized scales down to single-cell organelles, using various X-ray beam sizes. Our aim was to investigate the cellular uptake and distribution of Q [...] Read more.
X-ray fluorescence (XRF) imaging is a highly sensitive non-invasive imaging method for detection of small element quantities in objects, from human-sized scales down to single-cell organelles, using various X-ray beam sizes. Our aim was to investigate the cellular uptake and distribution of Q10, a highly conserved coenzyme with antioxidant and bioenergetic properties. Q10 was labeled with iodine (I2-Q10) and individual primary human skin cells were scanned with nano-focused beams. Distribution of I2-Q10 molecules taken up inside the screened individual skin cells was measured, with a clear correlation between individual Q10 uptake and cell size. Experiments revealed that labeling Q10 with iodine causes no artificial side effects as a result of the labeling procedure itself, and thus is a perfect means of investigating bioavailability and distribution of Q10 in cells. In summary, individual cellular Q10 uptake was demonstrated by XRF, opening the path towards Q10 multi-scale tracking for biodistribution studies. Full article
(This article belongs to the Special Issue The Ubiquitous and Multifaceted Coenzyme Q)
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Review

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22 pages, 2309 KiB  
Review
Biosynthesis, Deficiency, and Supplementation of Coenzyme Q
by Carmine Staiano, Laura García-Corzo, David Mantle, Nadia Turton, Lauren E. Millichap, Gloria Brea-Calvo and Iain Hargreaves
Antioxidants 2023, 12(7), 1469; https://doi.org/10.3390/antiox12071469 - 21 Jul 2023
Cited by 2 | Viewed by 3665
Abstract
Originally identified as a key component of the mitochondrial respiratory chain, Coenzyme Q (CoQ or CoQ10 for human tissues) has recently been revealed to be essential for many different redox processes, not only in the mitochondria, but elsewhere within other cellular membrane [...] Read more.
Originally identified as a key component of the mitochondrial respiratory chain, Coenzyme Q (CoQ or CoQ10 for human tissues) has recently been revealed to be essential for many different redox processes, not only in the mitochondria, but elsewhere within other cellular membrane types. Cells rely on endogenous CoQ biosynthesis, and defects in this still-not-completely understood pathway result in primary CoQ deficiencies, a group of conditions biochemically characterised by decreased tissue CoQ levels, which in turn are linked to functional defects. Secondary CoQ deficiencies may result from a wide variety of cellular dysfunctions not directly linked to primary synthesis. In this article, we review the current knowledge on CoQ biosynthesis, the defects leading to diminished CoQ10 levels in human tissues and their associated clinical manifestations. Full article
(This article belongs to the Special Issue The Ubiquitous and Multifaceted Coenzyme Q)
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19 pages, 1580 KiB  
Review
How the Disruption of Mitochondrial Redox Signalling Contributes to Ageing
by Beatriz Castejon-Vega, Mario D. Cordero and Alberto Sanz
Antioxidants 2023, 12(4), 831; https://doi.org/10.3390/antiox12040831 - 29 Mar 2023
Cited by 7 | Viewed by 3393
Abstract
In the past, mitochondrial reactive oxygen species (mtROS) were considered a byproduct of cellular metabolism. Due to the capacity of mtROS to cause oxidative damage, they were proposed as the main drivers of ageing and age-related diseases. Today, we know that mtROS are [...] Read more.
In the past, mitochondrial reactive oxygen species (mtROS) were considered a byproduct of cellular metabolism. Due to the capacity of mtROS to cause oxidative damage, they were proposed as the main drivers of ageing and age-related diseases. Today, we know that mtROS are cellular messengers instrumental in maintaining cellular homeostasis. As cellular messengers, they are produced in specific places at specific times, and the intensity and duration of the ROS signal determine the downstream effects of mitochondrial redox signalling. We do not know yet all the processes for which mtROS are important, but we have learnt that they are essential in decisions that affect cellular differentiation, proliferation and survival. On top of causing damage due to their capacity to oxidize cellular components, mtROS contribute to the onset of degenerative diseases when redox signalling becomes dysregulated. Here, we review the best-characterized signalling pathways in which mtROS participate and those pathological processes in which they are involved. We focus on how mtROS signalling is altered during ageing and discuss whether the accumulation of damaged mitochondria without signalling capacity is a cause or a consequence of ageing. Full article
(This article belongs to the Special Issue The Ubiquitous and Multifaceted Coenzyme Q)
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