Therapeutic Potential and Immunomodulatory Role of Coenzyme Q<sub>10</sub> and Its Analogues in Systemic Autoimmune Diseases

Coenzyme Q<sub>10</sub> (CoQ<sub>10</sub>) is a mitochondrial electron carrier and a powerful lipophilic antioxidant located in membranes and plasma lipoproteins. CoQ<sub>10</sub> is endogenously synthesized and obtained from the diet, which has raised interest in...

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Main Authors: Chary López-Pedrera, José Manuel Villalba, Alejandra Mª Patiño-Trives, Maria Luque-Tévar, Nuria Barbarroja, Mª Ángeles Aguirre, Alejandro Escudero-Contreras, Carlos Pérez-Sánchez
Format: Article
Language:English
Published: MDPI AG 2021-04-01
Series:Antioxidants
Subjects:
Online Access:https://www.mdpi.com/2076-3921/10/4/600
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author Chary López-Pedrera
José Manuel Villalba
Alejandra Mª Patiño-Trives
Maria Luque-Tévar
Nuria Barbarroja
Mª Ángeles Aguirre
Alejandro Escudero-Contreras
Carlos Pérez-Sánchez
author_facet Chary López-Pedrera
José Manuel Villalba
Alejandra Mª Patiño-Trives
Maria Luque-Tévar
Nuria Barbarroja
Mª Ángeles Aguirre
Alejandro Escudero-Contreras
Carlos Pérez-Sánchez
author_sort Chary López-Pedrera
collection DOAJ
description Coenzyme Q<sub>10</sub> (CoQ<sub>10</sub>) is a mitochondrial electron carrier and a powerful lipophilic antioxidant located in membranes and plasma lipoproteins. CoQ<sub>10</sub> is endogenously synthesized and obtained from the diet, which has raised interest in its therapeutic potential against pathologies related to mitochondrial dysfunction and enhanced oxidative stress. Novel formulations of solubilized CoQ<sub>10</sub> and the stabilization of reduced CoQ<sub>10</sub> (ubiquinol) have improved its bioavailability and efficacy. Synthetic analogues with increased solubility, such as idebenone, or accumulated selectively in mitochondria, such as MitoQ, have also demonstrated promising properties. CoQ<sub>10</sub> has shown beneficial effects in autoimmune diseases. Leukocytes from antiphospholipid syndrome (APS) patients exhibit an oxidative perturbation closely related to the prothrombotic status. In vivo ubiquinol supplementation in APS modulated the overexpression of inflammatory and thrombotic risk-markers. Mitochondrial abnormalities also contribute to immune dysregulation and organ damage in systemic lupus erythematosus (SLE). Idebenone and MitoQ improved clinical and immunological features of lupus-like disease in mice. Clinical trials and experimental models have further demonstrated a therapeutic role for CoQ<sub>10</sub> in Rheumatoid Arthritis, multiple sclerosis and type 1 diabetes. This review summarizes the effects of CoQ<sub>10</sub> and its analogs in modulating processes involved in autoimmune disorders, highlighting the potential of these therapeutic approaches for patients with immune-mediated diseases.
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spelling doaj.art-2f6821a71565469a9115d56628955b602023-11-21T15:26:31ZengMDPI AGAntioxidants2076-39212021-04-0110460010.3390/antiox10040600Therapeutic Potential and Immunomodulatory Role of Coenzyme Q<sub>10</sub> and Its Analogues in Systemic Autoimmune DiseasesChary López-Pedrera0José Manuel Villalba1Alejandra Mª Patiño-Trives2Maria Luque-Tévar3Nuria Barbarroja4Mª Ángeles Aguirre5Alejandro Escudero-Contreras6Carlos Pérez-Sánchez7Rheumatology Service, Reina Sofia Hospital/Maimonides Institute for Research in Biomedicine of Córdoba (IMIBIC), University of Córdoba, 14004 Córdoba, SpainDepartment of Cell Biology, Immunology and Physiology, Agrifood Campus of International Excellence, University of Córdoba, ceiA3, 14014 Córdoba, SpainRheumatology Service, Reina Sofia Hospital/Maimonides Institute for Research in Biomedicine of Córdoba (IMIBIC), University of Córdoba, 14004 Córdoba, SpainRheumatology Service, Reina Sofia Hospital/Maimonides Institute for Research in Biomedicine of Córdoba (IMIBIC), University of Córdoba, 14004 Córdoba, SpainRheumatology Service, Reina Sofia Hospital/Maimonides Institute for Research in Biomedicine of Córdoba (IMIBIC), University of Córdoba, 14004 Córdoba, SpainRheumatology Service, Reina Sofia Hospital/Maimonides Institute for Research in Biomedicine of Córdoba (IMIBIC), University of Córdoba, 14004 Córdoba, SpainRheumatology Service, Reina Sofia Hospital/Maimonides Institute for Research in Biomedicine of Córdoba (IMIBIC), University of Córdoba, 14004 Córdoba, SpainDepartment of Cell Biology, Immunology and Physiology, Agrifood Campus of International Excellence, University of Córdoba, ceiA3, 14014 Córdoba, SpainCoenzyme Q<sub>10</sub> (CoQ<sub>10</sub>) is a mitochondrial electron carrier and a powerful lipophilic antioxidant located in membranes and plasma lipoproteins. CoQ<sub>10</sub> is endogenously synthesized and obtained from the diet, which has raised interest in its therapeutic potential against pathologies related to mitochondrial dysfunction and enhanced oxidative stress. Novel formulations of solubilized CoQ<sub>10</sub> and the stabilization of reduced CoQ<sub>10</sub> (ubiquinol) have improved its bioavailability and efficacy. Synthetic analogues with increased solubility, such as idebenone, or accumulated selectively in mitochondria, such as MitoQ, have also demonstrated promising properties. CoQ<sub>10</sub> has shown beneficial effects in autoimmune diseases. Leukocytes from antiphospholipid syndrome (APS) patients exhibit an oxidative perturbation closely related to the prothrombotic status. In vivo ubiquinol supplementation in APS modulated the overexpression of inflammatory and thrombotic risk-markers. Mitochondrial abnormalities also contribute to immune dysregulation and organ damage in systemic lupus erythematosus (SLE). Idebenone and MitoQ improved clinical and immunological features of lupus-like disease in mice. Clinical trials and experimental models have further demonstrated a therapeutic role for CoQ<sub>10</sub> in Rheumatoid Arthritis, multiple sclerosis and type 1 diabetes. This review summarizes the effects of CoQ<sub>10</sub> and its analogs in modulating processes involved in autoimmune disorders, highlighting the potential of these therapeutic approaches for patients with immune-mediated diseases.https://www.mdpi.com/2076-3921/10/4/600coenzyme Q10autoimmune disordersinflammationcardiovascular disease
spellingShingle Chary López-Pedrera
José Manuel Villalba
Alejandra Mª Patiño-Trives
Maria Luque-Tévar
Nuria Barbarroja
Mª Ángeles Aguirre
Alejandro Escudero-Contreras
Carlos Pérez-Sánchez
Therapeutic Potential and Immunomodulatory Role of Coenzyme Q<sub>10</sub> and Its Analogues in Systemic Autoimmune Diseases
Antioxidants
coenzyme Q10
autoimmune disorders
inflammation
cardiovascular disease
title Therapeutic Potential and Immunomodulatory Role of Coenzyme Q<sub>10</sub> and Its Analogues in Systemic Autoimmune Diseases
title_full Therapeutic Potential and Immunomodulatory Role of Coenzyme Q<sub>10</sub> and Its Analogues in Systemic Autoimmune Diseases
title_fullStr Therapeutic Potential and Immunomodulatory Role of Coenzyme Q<sub>10</sub> and Its Analogues in Systemic Autoimmune Diseases
title_full_unstemmed Therapeutic Potential and Immunomodulatory Role of Coenzyme Q<sub>10</sub> and Its Analogues in Systemic Autoimmune Diseases
title_short Therapeutic Potential and Immunomodulatory Role of Coenzyme Q<sub>10</sub> and Its Analogues in Systemic Autoimmune Diseases
title_sort therapeutic potential and immunomodulatory role of coenzyme q sub 10 sub and its analogues in systemic autoimmune diseases
topic coenzyme Q10
autoimmune disorders
inflammation
cardiovascular disease
url https://www.mdpi.com/2076-3921/10/4/600
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