López-Pedrera, CharyVillalba, José ManuelPatiño-Trives, Alejandra MªLuque-Tévar, MariaBarbarroja, NuriaAguirre, Mª ÁngelesEscudero-Contreras, AlejandroPérez-Sánchez, Carlos2022-10-032022-10-032021-04-13López-Pedrera C, Villalba JM, Patiño-Trives AM, Luque-Tévar M, Barbarroja N, Aguirre MÁ, et al. Therapeutic Potential and Immunomodulatory Role of Coenzyme Q10 and Its Analogues in Systemic Autoimmune Diseases. Antioxidants. 2021 Apr 13;10(4):600.http://hdl.handle.net/10668/4208Coenzyme Q10 (CoQ10) is a mitochondrial electron carrier and a powerful lipophilic antioxidant located in membranes and plasma lipoproteins. CoQ10 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 CoQ10 and the stabilization of reduced CoQ10 (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. CoQ10 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 CoQ10 in Rheumatoid Arthritis, multiple sclerosis and type 1 diabetes. This review summarizes the effects of CoQ10 and its analogs in modulating processes involved in autoimmune disorders, highlighting the potential of these therapeutic approaches for patients with immune-mediated diseases.enAtribución 4.0 Internacionalhttp://creativecommons.org/licenses/by/4.0/Coenzyme Q10Autoimmune disordersInflammationCardiovascular diseaseAntiphospholipid syndromeMultiple sclerosisOxidative stressSystemic lupus erythematosusRheumatoid arthritisEnfermedades autoinmunes del sistema nerviosoInflamaciónEnfermedades cardiovascularesSíndrome antifosfolípidoEsclerosis múltipleEstrés oxidativoLupus eritematoso sistémicoArtritis reumatoideMedical Subject Headings::Organisms::Eukaryota::Animals::Chordata::Vertebrates::Mammals::Rodentia::Muridae::Murinae::MiceMedical Subject Headings::Organisms::Eukaryota::AnimalsMedical Subject Headings::Organisms::Eukaryota::Animals::Chordata::Vertebrates::Mammals::Primates::Haplorhini::Catarrhini::Hominidae::HumansMedical Subject Headings::Chemicals and Drugs::Chemical Actions and Uses::Pharmacologic Actions::Molecular Mechanisms of Pharmacological Action::AntioxidantsMedical Subject Headings::Diseases::Immune System Diseases::Autoimmune Diseases::Antiphospholipid SyndromeMedical Subject Headings::Phenomena and Processes::Metabolic Phenomena::Pharmacokinetics::Biological AvailabilityMedical Subject Headings::Diseases::Endocrine System Diseases::Diabetes Mellitus::Diabetes Mellitus, Type 1Medical Subject Headings::Phenomena and Processes::Physical Phenomena::Elementary Particles::ElectronsMedical Subject Headings::Diseases::Immune System Diseases::Autoimmune Diseases::Autoimmune Diseases of the Nervous System::Demyelinating Autoimmune Diseases, CNS::Multiple SclerosisMedical Subject Headings::Phenomena and Processes::Chemical Phenomena::Physicochemical Phenomena::SolubilityMedical Subject Headings::Anatomy::Cells::Cellular Structures::Intracellular Space::Cytoplasm::Cytoplasmic Structures::Organelles::MitochondriaMedical Subject Headings::Phenomena and Processes::Metabolic Phenomena::Metabolism::Oxidative StressMedical Subject Headings::Technology and Food and Beverages::Food and Beverages::Food::Dietary SupplementsMedical Subject Headings::Diseases::Skin and Connective Tissue Diseases::Connective Tissue Diseases::Lupus Erythematosus, SystemicMedical Subject Headings::Diseases::Musculoskeletal Diseases::Rheumatic Diseases::Arthritis, RheumatoidMedical Subject Headings::Analytical, Diagnostic and Therapeutic Techniques and Equipment::Investigative Techniques::Models, TheoreticalMedical Subject Headings::Chemicals and Drugs::Lipids::LipoproteinsMedical Subject Headings::Anatomy::Hemic and Immune Systems::Immune System::LeukocytesMedical Subject Headings::Phenomena and Processes::Physiological Phenomena::Nutritional Physiological Phenomena::Dietreview article33924642open access10.3390/antiox100406002076-3921PMC8069673