Coenzyme Q10: Role in Less Common Age-Related Disorders

Abstract

In this article we have reviewed the potential role of coenzyme Q10 (CoQ10) in the pathogenesis and treatment of a number of less common age-related disorders, for many of which effective therapies are not currently available. For most of these disorders, mitochondrial dysfunction, oxidative stress and inflammation have been implicated in the disease process, providing a rationale for the potential therapeutic use of CoQ10, because of its key roles in mitochondrial function, as an antioxidant, and as an anti-inflammatory agent. Disorders reviewed in the article include multi system atrophy, progressive supranuclear palsy, sporadic adult onset ataxia, and pulmonary fibrosis, together with late onset versions of Huntington’s disease, Alexander disease, lupus, anti-phospholipid syndrome, lysosomal storage disorders, fibromyalgia, Machado-Joseph disease, acyl-CoA dehydrogenase deficiency, and Leber’s optic neuropathy.

Genetics of Multiple System Atrophy and Progressive Supranuclear Palsy: A Systemized Review of the Literature

“…COQ2 produces the enzyme coenzyme-Q2-polyprenyltransferase in the biosynthesis of coenzyme Q 10 , which serves to transport electrons from complexes I and II to complex III [ 32 ]. Deficits in coenzyme Q 10 impair oxidative phosphorylation and enhance the vulnerability of cells to harm by free radicals [ 32 ].…”

“…COQ2 produces the enzyme coenzyme-Q2-polyprenyltransferase in the biosynthesis of coenzyme Q 10 , which serves to transport electrons from complexes I and II to complex III [ 32 ]. Deficits in coenzyme Q 10 impair oxidative phosphorylation and enhance the vulnerability of cells to harm by free radicals [ 32 ]. In a recent meta-analysis, V393A was shown to be a susceptibility variant instead of causative for MSA (specifically, MSA-C) in East Asian cohorts [ 33 ].…”