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
Source: PubMed


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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    • "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; 2014:793879. 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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    • "CoQ 10 is a lipid-soluble compound located in the inner mitochondrial membrane where it functions as a part of the electron transport chain as well as a strong endogenous lipophilic antioxidant (Turunen et al. 2004). CoQ 10 is commonly used as a dietary supplement , with the rationale that increasing intake of the nutrient boosts cellular metabolism, particularly in cells with energy deficiencies (Mancuso et al. 2010). The effect of CoQ 10 has been tested on various diseases such as diabetes (Hodgson et al. 2002), myocardial infarction (Singh et al. 1998; Soja and Mortensen 1997), angina (Kogan et al. 1999), and Parkinson's disease (Shults et al. 2004), as well as in anti-breast cancer clinical trials (Portakal et al. 2000). "
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    ABSTRACT: Recently, there has been a growing demand for therapeutic monoclonal antibodies (MAbs) on the global market. Because therapeutic MAbs are more expensive than low-molecular-weight drugs, there have been strong demands to lower their production costs. Therefore, efficient methods to minimize the cost of goods are currently active areas of research. We have screened several enhancers of specific MAb production rate (SPR) using a YB2/0 cell line and found that coenzyme-Q(10) (CoQ(10)) is a promising enhancer candidate. CoQ(10) is well known as a strong antioxidant in the respiratory chain and is used for healthcare and other applications. Because CoQ(10) is negligibly water soluble, most studies are limited by low concentrations. We added CoQ(10) to a culture medium as dispersed nanoparticles at several concentrations (Q-Media) and conducted a fed-batch culture. Although the Q-Media had no effect on cumulative viable cell density, it enhanced SPR by 29%. In addition, the Q-Media had no effect on the binding or cytotoxic activity of MAbs. Q-Media also enhanced SPR with CHO and NS0 cell lines by 30%. These observations suggest that CoQ(10) serves as a powerful aid in the production of MAbs by enhancing SPR without changing the characteristics of cell growth, or adversely affecting the quality or biological activity of MAbs.
    Cytotechnology 03/2011; 63(2):163-70. DOI:10.1007/s10616-010-9330-9 · 1.75 Impact Factor
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