Coenzyme Q10 in Neuromuscular and Neurodegenerative Disorders

Department of Neuroscience, Neurological Clinic, University of Pisa, Italy.
Current drug targets (Impact Factor: 3.02). 01/2010; 11(1):111-21. DOI: 10.2174/138945010790031018
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Coenzyme Q10 (CoQ10, or ubiquinone) is an electron carrier of the mitochondrial respiratory chain (electron transport chain) with antioxidant properties. In view of the involvement of CoQ10 in oxidative phosphorylation and cellular antioxidant protection a deficiency in this quinone would be expected to contribute to disease pathophysiology by causing a failure in energy metabolism and antioxidant status. Indeed, a deficit in CoQ10 status has been determined in a number of neuromuscular and neurodegenerative disorders. Primary disorders of CoQ10 biosynthesis are potentially treatable conditions and therefore a high degree of clinical awareness about this condition is essential. A secondary loss of CoQ10 status following HMG-Coa reductase inhibitor (statins) treatment has be implicated in the pathophysiology of the myotoxicity associated with this pharmacotherapy. CoQ10 and its analogue, idebenone, have been widely used in the treatment of neurodegenerative and neuromuscular disorders. These compounds could potentially play a role in the treatment of mitochondrial disorders, Parkinson's disease, Huntington's disease, amyotrophic lateral sclerosis, Friedreich's ataxia, and other conditions which have been linked to mitochondrial dysfunction. This article reviews the physiological roles of CoQ10, as well as the rationale and the role in clinical practice of CoQ10 supplementation in different neurological and muscular diseases, from primary CoQ10 deficiency to neurodegenerative disorders. We also briefly report a case of the myopathic form of CoQ10 deficiency.

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    • "J. Castro-Marrero et al. / Clinical Nutrition xxx (2015) 1e9 6 Please cite this article in press as: Castro-Marrero J, et al., Effect of coenzyme Q 10 plus nicotinamide adenine dinucleotide supplementation on maximum heart rate after exercise testing in chronic fatigue syndrome e A randomized, controlled, double-blind trial, Clinical Nutrition (2015), associated with neurodegenerative diseases and cancer [29] [30]. CoQ 10 and NADH deficiencies have also been described in patients with CFS and fibromyalgia [9] [12] [31], and it has also been suggested that serum NADH levels are directly correlated with serum CoQ 10 concentration in these patients. "
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    ABSTRACT: Chronic Fatigue Syndrome (CFS) is a complex condition, characterized by severe disabling fatigue with no known cause, no established diagnostic tests, and no universally effective treatment. Several studies have proposed symptomatic treatment with coenzyme Q10 (CoQ10) and nicotinamide adenine dinucleotide (NADH) supplementation. The primary endpoint was to assess the effect of CoQ10 plus NADH supplementation on age-predicted maximum heart rate (max HR) during a cycle ergometer test. Secondary measures included fatigue, pain and sleep. A proof-of-concept, 8-week, randomized, controlled, double-blind trial was conducted in 80 CFS patients assigned to receive either CoQ10 plus NADH supplementation or matching placebo twice daily. Maximum HR was evaluated at baseline and at end of the run-in period using an exercise test. Fatigue, pain and sleep were evaluated at baseline, and then reassessed at 4- and 8-weeks through self-reported questionnaires. The CoQ10 plus NADH group showed a significant reduction in max HR during a cycle ergometer test at week 8 versus baseline (P = 0.022). Perception of fatigue also showed a decrease through all follow-up visits in active group versus placebo (P = 0.03). However, pain and sleep did not improve in the active group. Coenzyme Q10 plus NADH was generally safe and well tolerated. Our results suggest that CoQ10 plus NADH supplementation for 8 weeks is safe and potentially effective in reducing max HR during a cycle ergometer test and also on fatigue in CFS. Further additional larger controlled trials are needed to confirm these findings. Clinical trial registrationThis trial was registered at as NCT02063126. Copyright © 2015 The Authors. Published by Elsevier Ltd.. All rights reserved.
    Clinical nutrition (Edinburgh, Scotland) 07/2015; 121. DOI:10.1016/j.clnu.2015.07.010 · 4.48 Impact Factor
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    • "It is an efficient antioxidant against free radicals and lipid peroxidation [1–3]. Many studies have reported CoQ10 deficiencies among patients with cardiovascular disease [4], neurodegenerative disorders [5], diabetes [6], statin-associated myopathy [7], and cancer [8]. Supplementation with CoQ10 has proven beneficial in treating these diseases, and numerous clinical trials are investigating its use as a drug or dietary supplement [8]. "
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    ABSTRACT: To improve the bioavailability of orally administered lipophilic coenzyme Q10 (CoQ10), we formulated a novel lipid-free nano-CoQ10 system stabilized by various surfactants. Nano-CoQ10s, composed of 2.5% (w/w) CoQ10, 1.67% (w/w) surfactant, and 41.67% (w/w) glycerol, were prepared by hot high-pressure homogenization. The resulting formulations were characterized by particle size, zeta potential, differential scanning calorimetry, and cryogenic transmission electron microscopy. We found that the mean particle size of all nano-CoQ10s ranged from 66.3 ± 1.5 nm to 92.7 ± 1.5 nm and the zeta potential ranged from -12.8 ± 1.4 mV to -41.6 ± 1.4 mV. The CoQ10 in nano-CoQ10s likely existed in a supercooled state, and nano-CoQ10s stored in a brown sealed bottle were stable for 180 days at 25°C. The bioavailability of CoQ10 was evaluated following oral administration of CoQ10 formulations in Sprague-Dawley rats. Compared to the values observed following administration of CoQ10-Suspension, nano-CoQ10 modified with various surfactants significantly increased the maximum plasma concentration and the area under the plasma concentration-time curve. Thus, the lipid-free system of a nano-CoQ10 stabilized with a surfactant may be an effective vehicle for improving oral bioavailability of CoQ10.
    BioMed Research International 06/2014; 2014(1):793879. DOI:10.1155/2014/793879 · 2.71 Impact Factor
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    • "Free radical stress, inflammation and mitochondrial damage as well as abnormality in energy metabolism have been observed in neurodegenerative diseases [6] [7] [8] [9] [10] [11] [12] [13]. The neurons in the brain and spinal cord are vulnerable to free radical attack, because they are highly oxygenated structures and contain large amount of iron and polyunsaturated fatty acids and poor antioxidant systems [3] [4] [5] [6] [7] [8]. Deficiency of antioxidants, particularly CoQ10 in the neurons may increase the vulnerability of neuronal membrane mitochondria to damage resulting into degeneration. "

    The Open Nutraceuticals Journal 10/2012; 5(1):187-192. DOI:10.2174/1876396001205010187
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