Short-term effects of coenzyme Q(10) in progressive supranuclear palsy: A randomized, placebo-controlled trial
Department of Neurology, Philipps University, Marburg, Germany. Movement Disorders
(Impact Factor: 5.68).
05/2008; 23(7):942-9. DOI: 10.1002/mds.22023
Mitochondrial complex I appears to be dysfunctional in progressive supranuclear palsy (PSP). Coenzyme Q(10) (CoQ(10)) is a physiological cofactor of complex I. Therefore, we evaluated the short-term effects of CoQ(10) in PSP. We performed a double-blind, randomized, placebo-controlled, phase II trial, including 21 clinically probable PSP patients (stage < or = III) to receive a liquid nanodispersion of CoQ(10) (5 mg/kg/day) or matching placebo. Over a 6-week period, we determined the change in CoQ(10) serum concentration, cerebral energy metabolites (by (31)P- and (1)H-magnetic resonance spectroscopy), motor and neuropsychological dysfunction (PSP rating scale, UPDRS III, Hoehn and Yahr stage, Frontal Assessment Battery, Mini Mental Status Examination, Montgomery Asberg Depression Scale). CoQ(10) was safe and well tolerated. In patients receiving CoQ(10) compared to placebo, the concentration of low-energy phosphates (adenosine-diphosphate, unphosphorylated creatine) decreased. Consequently, the ratio of high-energy phosphates to low-energy phosphates (adenosine-triphosphate to adenosine-diphosphate, phospho-creatine to unphosphorylated creatine) increased. These changes were significant in the occipital lobe and showed a consistent trend in the basal ganglia. Clinically, the PSP rating scale and the Frontal Assessment Battery improved slightly, but significantly, upon CoQ(10) treatment compared to placebo. Since CoQ(10) appears to improve cerebral energy metabolism in PSP, long-term treatment might have a disease-modifying, neuroprotective effect.
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Available from: Asako Yoritaka
- "In a trial for PSP, CoQ 10 improved cerebral energy metabolism, which correlated with an improvement of symptoms . This implies that Ubiquinol-10 might improve metabolism in the dopaminergic neurons remaining in patients with PD. "
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ABSTRACT: Mitochondrial complex I deficiencies have been found in post-mortem brains of patients with Parkinson's disease (PD). Coenzyme Q10 (CoQ10) is the electron acceptor found in complexes I and II, and is a potent antioxidant. A recent trial of the oxidized form of CoQ10 for PD failed to show benefits; however, the reduced form of CoQ10 (ubiquinol-10) has shown better neuroprotective effects in animal models.
Randomized, double-blind, placebo-controlled, parallel-group pilot trials were conducted to assess the efficacy of ubiquinol-10 in Japanese patients with PD. Participants were divided into two groups: PD experiencing wearing off (Group A), and early PD, without levodopa (with or without a dopamine agonist) (Group B). Participants took 300 mg of ubiquinol-10 or placebo per day for 48 weeks (Group A) or 96 weeks (Group B).
In Group A, total Unified Parkinson's Disease Rating Scale (UPDRS) scores decreased in the ubiquinol-10 group (n = 14; mean ± SD [-4.2 ± 8.2]), indicating improvement in symptoms. There was a statistically significant difference (p < 0.05) compared with the placebo group (n = 12; 2.9 ± 8.9). In Group B, UPDRS increased in the ubiquinol-10 group (n = 14; 3.9 ± 8.0), as well as in the placebo group (n = 8; 5.1 ± 10.3).
This is the first report showing that ubiquinol-10 may significantly improve PD with wearing off, as judged by total UPDRS scores, and that ubiquinol-10 is safe and well tolerated.
Copyright © 2015 Elsevier Ltd. All rights reserved.
Parkinsonism & Related Disorders 05/2015; 21(8). DOI:10.1016/j.parkreldis.2015.05.022 · 3.97 Impact Factor
Available from: Mario D Cordero
- "A large Phase III trial comparing placebo and 1200 mg and 2400 mg of CoQ 10 daily is currently under way. A recent magnetic resonance spectroscopic study also was conducted in patients with progressive supranuclear palsy treated with CoQ 10 ; a significant increase of the ratio of high-energy to low-energy phosphates was indicative of improved oxidative phosphorylation of the occipital cortex . "
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ABSTRACT: Fibromyalgia (FM) is a chronic pain syndrome with unknown etiology. Recent studies have shown evidence demonstrating that mitochondrial dysfunction and oxidative stress may have a role in the pathophysiology of FM. Coenzyme Q10 (CoQ10) is an essential electron carrier in the mitochondrial respiratory chain and a strong antioxidant. Low CoQ10 levels have been detected in patients with FM, and a significant decrease of clinical symptoms has been reported after oral CoQ10 supplementation. In this report, we show the effect of CoQ10 treatment on clinical symptoms, blood mononuclear cells, and mitochondrial and oxidative stress markers from a woman with FM. After CoQ10 treatment, the patient reported a significant improvement of clinical symptoms. At the cellular level, CoQ10 treatment restored mitochondrial dysfunction and the mtDNA copy number, decreased oxidative stress, and increased mitochondrial biogenesis. Our results suggest that CoQ10 could be an alternative therapeutic approach for FM.
Nutrition 08/2012; 28(11-12):1200-3. DOI:10.1016/j.nut.2012.03.018 · 2.93 Impact Factor
Available from: ncbi.nlm.nih.gov
- "First, very few pediatric subjects were enrolled in these studies; thus, the chronic safety and efficacy of CoQ 10 has never been examined prospectively in children. Second, the doses of CoQ 10 employed in the earlier trials were markedly lower (~2 mg/kg/d for a 70 kg adult) than those used in more recent studies, including those involving neurodegenerative disorders other than genetic mitochondrial diseases (Glover et al. 2010; Kaufmann et al. 2009; Mancuso et al. 2010; Stamelou et al. 2008; The Huntington Study Group 2001; The Huntington Study Group 2010). Third, the earlier trials of CoQ 10 provided evidence of its safety, at least in adults. "
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ABSTRACT: Although mitochondrial disease research in general is robust, adequate treatment of these life-threatening conditions has lagged, partly because of a persistence of clinical anecdotes as substitutes for scientifically and ethically rigorous clinical trials. Here I summarize the key lessons learned from some of the "first generation" of randomized controlled trials for genetic mitochondrial diseases and suggest how future trials may benefit from both past experience and exciting new resources available for patient-oriented research and training in this field.
Mitochondrion 05/2011; 11(5):679-85. DOI:10.1016/j.mito.2011.05.002 · 3.25 Impact Factor
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