Coenzyme Q at the Hinge of Health and Metabolic Diseases

Abstract

Coenzyme Q is a unique lipidic molecule highly conserved in evolution and essential to maintaining aerobic metabolism. It is endogenously synthesized in all cells by a very complex pathway involving a group of nuclear genes that share high homology among species. This pathway is tightly regulated at transcription and translation, but also by environment and energy requirements. Here, we review how coenzyme Q reacts within mitochondria to promote ATP synthesis and also integrates a plethora of metabolic pathways and regulates mitochondrial oxidative stress. Coenzyme Q is also located in all cellular membranes and plasma lipoproteins in which it exerts antioxidant function, and its reaction with different extramitochondrial oxidoreductases contributes to regulate the cellular redox homeostasis and cytosolic oxidative stress, providing a key factor in controlling various apoptosis mechanisms. Coenzyme Q levels can be decreased in humans by defects in the biosynthesis pathway or by mitochondrial or cytosolic dysfunctions, leading to a highly heterogeneous group of mitochondrial diseases included in the coenzyme Q deficiency syndrome. We also review the importance of coenzyme Q levels and its reactions involved in aging and age-associated metabolic disorders, and how the strategy of its supplementation has had benefits for combating these diseases and for physical performance in aging.

Levels of Plasma Coenzyme Q10 Are Associated with Physical Capacity and Cardiovascular Risk in the Elderly

“…Accordingly, in humans, the combination of exercise with CoQ 10 has been suggested to counteract sarcopenia in the elderly [ 55 ]. For this reason, CoQ 10 supplementation has been be suggested to decrease many dysfunctions associated with aging and age-related diseases [ 9 , 56 , 57 ].…”

“…Because of these essential activities, CoQ 10 can be consider a key component for the physiology of cells that play an important role in the progression of aging [ 1 , 8 , 9 ]. In fact, decreasing levels of CoQ 10 have been associated with the dysfunction of several organs in aged people.…”

“…In fact, decreasing levels of CoQ 10 have been associated with the dysfunction of several organs in aged people. CoQ is synthesized by a synthome comprising at least 11 different proteins acting between the endoplasmic reticulum and mitochondria [ 9 ]. The inability of cells to synthesize CoQ produces many severe symptoms, mainly affecting the cerebellum, the kidneys, and muscle [ 10 ].…”

“…CoQ 10 can be obtained from the diet although the amount obtained seems to be very low from this source. In blood plasma, CoQ 10 levels have been studied in aging to determine whether they can be considered to be aging-related biomarkers [ 9 , 13 ].…”

Rational Engineering of Non-Ubiquinone Containing Corynebacterium glutamicum for Enhanced Coenzyme Q10 Production

“…Next to its function in respiratory chains, CoQ serves as a lipid-soluble antioxidant that protects cellular membranes and lipoproteins from oxidative damage [ 1 ], as an activator of mitochondrial uncoupling proteins [ 2 ], and as a cofactor of several important enzymes such as mitochondrial dehydrogenases involved in different metabolic pathways [ 3 ]. Genetic CoQ 10 deficiencies may cause various severe disorders, of which symptoms can sometimes be mitigated by CoQ 10 supplementation [ 4 , 5 , 6 ]. Dietary supplementation has also shown beneficial effects in patients with cardiovascular and neurodegenerative diseases [ 7 , 8 ].…”