Disorders of Human Coenzyme Q10 Metabolism: An Overview

Abstract

Coenzyme Q10 (CoQ10) has a number of vital functions in all cells, both mitochondrial and extramitochondrial. In addition to its key role in mitochondrial oxidative phosphorylation, CoQ10 serves as a lipid soluble antioxidant, plays an important role in fatty acid, pyrimidine and lysosomal metabolism, as well as directly mediating the expression of a number of genes, including those involved in inflammation. In view of the central role of CoQ10 in cellular metabolism, it is unsurprising that a CoQ10 deficiency is linked to the pathogenesis of a range of disorders. CoQ10 deficiency is broadly classified into primary or secondary deficiencies. Primary deficiencies result from genetic defects in the multi-step biochemical pathway of CoQ10 synthesis, whereas secondary deficiencies can occur as result of other diseases or certain pharmacotherapies. In this article we have reviewed the clinical consequences of primary and secondary CoQ10 deficiencies, as well as providing some examples of the successful use of CoQ10 supplementation in the treatment of disease.

Does Coenzyme Q10 Supplementation Improve Testicular Function and Spermatogenesis in Male Mice with Chronic Kidney Disease?

“…Generally, CoQ10 is produced in tissues such as the liver, heart, kidney and skeletal muscle [ 17 ], and the major routes of excretion are in urine and feces [ 18 ]. Under normal conditions, endogenous CoQ10 satisfies a high proportion of the daily requirement [ 19 ], resulting in the excess being eliminated, which corresponds to the similar blood and tissue CoQ10 levels in the SC and SQ groups. Moreover, decreased CoQ10 levels have been observed in patients with CKD [ 20 ] and those with CKD undergoing dialysis [ 21 , 22 ], indicating an insufficient CoQ10 status during disease progression; therefore, CoQ10 treatment may be a potential therapy for CKD patients.…”

Ferroptosis Mechanisms Involved in Neurodegenerative Diseases

“…Moreover, the authors described that the antiferroptotic function of FSP1 is independent of cellular glutathione concentration, GPx4 activity, ACSL4 expression and oxidizable fatty acid content [ 79 ]. Coenzyme Q10 is an endogenous lipophilic antioxidant produced in the mevalonate pathway, as well as a part of the mitochondrial respiratory chain, and from the metabolism of fatty acid and pyrimidine [ 80 , 81 ]. Indeed, the homologous proteins MDM2 and MDMX, negative regulators of the tumor suppressor p53, promote ferroptosis by regulating lipid peroxidation by altering PPARα activity.…”

Effect of Dietary Coenzyme Q10 Plus NADH Supplementation on Fatigue Perception and Health-Related Quality of Life in Individuals with Myalgic Encephalomyelitis/Chronic Fatigue Syndrome: A Prospective, Randomized, Double-Blind, Placebo-Controlled Trial

“…Long-chain polyunsaturated fatty acid, prostaglandin and amino acid metabolism, and inefficient ATP biosynthesis have been suggested as consequences of ME/CFS due to perturbed mitochondrial bioenergetic metabolism [ 13 ]. Due to the potential role of the mitochondria in ME/CFS, mitochondrial-targeting nutraceutical interventions have been used to assist in improving patient outcomes such as fatigue and their health-related quality of life [ 14 ], including CoQ10, NADH, and n-Acetyl-L-carnitine as part of their treatment regime [ 15 , 16 , 17 , 18 ]. These treatments are administered either alone or in combination with a cocktail of other nutraceutical and/or pharmaceutical-based products.…”