Abstract
Publisher Summary This chapter analyzes the therapeutic effects of coenzyme Q 10 (CoQ 10 ) in neurodegenerative diseases. There is increasing interest in the potential usefulness of CoQ 10 to treat both mitochondrial disorders as well as neurodegenerative diseases, such as Parkinson's disease (PD), Huntington's disease (HD), and amyotrophic lateral sclerosis (ALS). CoQ 10 administration can increase brain concentrations in mature and older animals. It can also increase brain mitochondrial concentrations. There is substantial evidence that CoQ 10 can act in concert with alphatocopherol as an antioxidant within mitochondria. CoQ 10 administration has been demonstrated to be efficacious in experimental models of neurodegenerative diseases. It is neuroprotective against lesions produced by mitochondrial toxins including malonate, 3-nitropropionic acid and MPTP. CoQ 10 extends survival in a transgenic mouse model of ALS, and it extends survival and exerts significant neuroprotective effects in a transgenic mouse model of HD. CoQ 10 and idebenone appear to be promising for treatment of Friedriech's ataxia. Initial clinical trials in both HD and PD have shown beneficial effects. The initial results of utilizing CoQ 10 administration for treatment of neurodegenerative disease appear promising as a treatment to slow the inexorable progression of these disorders.
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