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Pharmaceuticals 2009, 2(3), 134-149; doi:10.3390/ph203134

Coenzyme Q10 and Neurological Diseases

* , , ,  and
Department of Neuroscience, Neurological Clinic, University of Pisa, Tuscany, Italy
* Author to whom correspondence should be addressed.
Received: 29 September 2009 / Revised: 26 November 2009 / Accepted: 30 November 2009 / Published: 1 December 2009
(This article belongs to the Special Issue Mitochondrial Drugs for Neurodegenerative Diseases)
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Coenzyme Q10 (CoQ10, or ubiquinone) is a small electron carrier of the mitochondrial respiratory chain with antioxidant properties. CoQ10 supplementation has been widely used for mitochondrial disorders. The rationale for using CoQ10 is very powerful when this compound is primary decreased because of defective synthesis. Primary CoQ10 deficiency is a treatable condition, so heightened “clinical awareness” about this diagnosis is essential. CoQ10 and its analogue, idebenone, have also been widely used in the treatment of other neurodegenerative disorders. These compounds could potentially play a therapeutic role in 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 diseases, from primary CoQ10 deficiency to neurodegenerative disorders.
Keywords: coenzyme Q10; idebenone; mitochondria; mitochondrial diseases; neurodegeneration coenzyme Q10; idebenone; mitochondria; mitochondrial diseases; neurodegeneration
This is an open access article distributed under the Creative Commons Attribution License (CC BY) which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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Mancuso, M.; Orsucci, D.; Calsolaro, V.; Choub, A.; Siciliano, G. Coenzyme Q10 and Neurological Diseases. Pharmaceuticals 2009, 2, 134-149.

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