Coenzyme Q10 in neurodegenerative disorders: Potential benefit of CoQ10 supplementation for multiple system atrophy

Abstract

Coenzyme Q10 (CoQ10) is an essential cofactor in the mitochondrial respiratory pathway and also functions as a lipid-soluble antioxidant. CoQ10 deficiency has been implicated in many clinical disorders and aging. Primary CoQ10 deficiency is a group of recessively inherited diseases caused by mutations in any gene involved in the CoQ10 biosynthesis pathway. Although primary CoQ10 deficiency is rare, its diagnosis is important because it is potentially treatable with exogenous CoQ10. Multiple system atrophy (MSA) was recently shown to be linked to mutations in the COQ2 gene, one of the genes involved in the CoQ10 biosynthesis pathway. MSA is relatively common in adult-onset neurodegenerative diseases characterized by Parkinsonism, cerebellar ataxia and autonomic failures. Because COQ2 mutations are associated with an increased risk of MSA, oral CoQ10 supplementation may be beneficial for MSA, as for other primary CoQ10 deficiencies. Statins are 3-hydroxy-3-methylglutaryl coenzyme A inhibitors that inhibit the biosynthesis of cholesterol, as well as the synthesis of mevalonate, a critical intermediate in cholesterol synthesis. Statin therapy has been associated with a variety of muscle complaints from myalgia to rhabdomyolysis. Statin treatment carries a potential risk of CoQ10 deficiency, although no definite evidence has implicated CQ10 deficiency as the cause of statin-related myopathy.

Keywords: Primary coenzyme Q10 deficiency, Multiple system atrophy, Cerebellar ataxia, COQ2 gene, Statin, Coenzyme Q10 supplementation, Reduced coenzyme Q10

Core tip: Recently, multiple system atrophy (MSA), relatively common in adult-onset neurodegenerative diseases, was shown to be linked to mutations in the COQ2 gene, one of the genes involved in the Coenzyme Q10 (CoQ10) biosynthesis pathway. Neurologists so far have not paid much attention to CoQ10 because primary CoQ10 deficiency caused by mutations in the CoQ10 synthesizing genes is very rare. The most important message is that primary CoQ10 deficiency is treatable with exogenous CoQ10 and that oral CoQ10 supplementation might be also beneficial for patients with MSA.