Abstract
Coenzyme 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.
Highlights
Introduction to Coenzyme Q and CoenzymeQ-Related Compounds1.1
Thereafter, through the implementation of a prospective, randomized, double-masked, placebo-controlled study (Clinical Trials.gov: NCT02218476), we evaluated the in vivo effects of ubiquinol supplementation to the standard therapy of APS patients on parameters related to thrombosis and inflammation
A role for mitochondrial dysfunction has been further proposed in the immune dysregulation and organ damage characteristic of autoimmune diseases
Summary
Coenzyme Q (CoQ) is a prenylated para-benzoquinone that is found ubiquitously in aerobic organisms and is present in all cells and tissues and in plasma lipoproteins, its denomination as ubiquinone [1,2]. CoQ participates in a trans-plasma membrane redox system that has been related to the control of cell growth, the reoxidation of cellular nicotinamide adenine dinucleotide (NADH) under conditions of mitochondrial dysfunction, and the regeneration of ascorbate in the extracellular space [8,9]. Another prominent function of CoQ (in its reduced form) is to provide antioxidant protection within the lipid phase of membranes and plasma lipoproteins, either directly or through the regeneration of α-tocopherol [3,7,8,9]. By the action of some pharmacological agents such as statins [20]
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