Coenzyme Q₁₀ in the Treatment of Mitochondrial Disease

Abstract

Currently, there is a paucity of available treatment strategies for oxidative phosphorylation disorders. Coenzyme Q 10 (CoQ 10 ) and related synthetic quinones are the only agents to date that have proven to be beneficial in the treatment of these heterogeneous disorders. The therapeutic efficacy of CoQ 10 is not restricted to patients with an underlying CoQ 10 deficiency and is thought to result from its ability to restore electron flow in the mitochondrial respiratory chain (MRC) as well as to increase the cellular antioxidant capacity. At present, however, there is no consensus on the appropriate dosage or therapeutic plasma level of CoQ 10 , and this information will be required before CoQ 10 can be utilized effectively in the treatment of mitochondrial disease. The following review will outline our current knowledge on the use of CoQ 10 in the treatment of MRC disorders and primary CoQ 10 deficiencies.

Chronic kidney disease and coenzyme Q10 supplementation

“…These enzymes are NADH cytochrome b5 reductase, NADH/NADPH oxidoreductase, NADPH coenzyme Q reductase (NQO1) and dihydro-orotate dehydrogenase (NQO1; Villalba & Navas 2000;Takahashi et al 1996). In addition to their antioxidant potential however, the therapeutic efficacy of CoQ10 and its synthetic analogues such idebenone in the treatment of MRC disorders is also thought to rely on their ability to enhance electron flow in the MRC (Hargreaves, 2014;Neergheen et al, 2017).…”

Ubiquinol treatment for TBI in male rats: Effects on mitochondrial integrity, injury severity, and neurometabolism

“…After TBI, the brain mitochondria are vulnerable to secondary injury, and there is loss of cellular function due to reactive oxygen species. Ubiquinol is naturally produced in cell mitochondria, is essential for adenosine triphosphate (ATP) synthesis, and increases the cellular antioxidant capacity (Lu et al, ; Neergheen et al, ). We administered ubiquinol before and after TBI, and demonstrated a significant reduction in programmed cell death, mitochondrial damage, and serum biomarkers of TBI severity.…”

Understanding the role of OXPHOS dysfunction in the pathogenesis of ECHS1 deficiency

“…These treatments are mostly nonspecific and include dietary changes, exercise‐related therapies or mitochondrial ‘cocktails’ that contain CoQ 10 , vitamins C and E, riboflavine, creatine monohydrate and other antioxidants . These treatments can help alleviate disease symptoms; however, only anecdotal evidence currently exists to support their use , with a Cochrane review finding no clear evidence to support the use of any current mitochondrial disease treatments .…”