Coenzyme Q serves as the linking molecule between different metabolic pathways. Indeed, this essential endogenously synthesized molecule is the cornerstone of many energy production pathways. Furthermore, its location in the inner mitochondrial membrane and its antioxidant capacity protect against oxidative stress. Coenzyme Q supplementation improves essential mitochondrial functions protecting against aging-associated diseases.

Coenzyme Q Biosynthesis:

The coenzyme Q molecule has two central parts; the first is a benzoquinone ring, while the second is a long polyisoprenoid lipid chain. Consequently, the chemical characteristics of its composition provide coenzyme Q with different capabilities. For instance, the benzoquinone ring has redox sites that allow coenzyme Q’s antioxidant properties. In addition, its lipid chain provides coenzymes Q’s beneficial location at the mitochondrial inner membrane; therefore, its function in the electron transport chain.

Coenzyme Q’s synthesis is not fully elucidated and has many different synthesis pathways being studied at present. Nevertheless, while some researchers suggest that CoQ synthesizes in the mitochondria, others indicate that CoQ has its synthesis driven by the endoplasmic reticulum or depends on extramitochondrial pools of CoQ.

• Furthermore, the structural biosynthesis of CoQ divides into four stages: 

1. Quinone synthesis: Dependent on benzoquinone as the primary aromatic precursor, utilizing phenylalanine or tyrosine as ring precursors. In addition, other aromatic ring precursors can incorporate into CoQ’s synthesis in the presence of a genetic mutation. These precursors can be 2,4-dihydroxybenzoic acid (2,4-diHB), 3,4-dihydroxybenzoic acid (3,4-diHB), and vanillic acid.
2. Isoprenoid tail synthesis: The addition of dimethylally pyrophosphate (DMAPP) and isopentenyl pyrophosphate (IPP) creates decaprenyl diphosphate and nonaprenyl diphosphate. The precursors of this synthesis come from the mevalonate pathway.
3. Condensation of both molecules (quinone and isoprenoid): The attachment between the ring structure and the isoprenoid tail is catalyzed by CoQ2.
4. Ring modification: Finally, this condensed molecule undergoes ring modification through various reactions of methylations, decarboxylation, and hydroxylations which lead to the generation of CoQ9 or CoQ10.

Different enzymes modulate these reactions. Among them, enzymes from the flavin-dependent monooxygenase family come into play and S-adenosyl methionine (SAM)-dependent methyltransferase.

Pathogenic gene mutations affecting CoQ synthesis:

There are several autosomal recessive mutations in CoQ genes that can cause primary ubiquinone deficiency. Furthermore, these mutations can affect patients and show signs and symptoms like fatigue, muscle weakness, learning disabilities, seizures, encephalopathy, epilepsy, dystonia, and cardiomyopathy. 

Some of the identified pathogenic gene mutations associated with these mitochondrial disease symptoms are:

• PDSS1 and PDSS2
• CoQ2
• CoQ6
• CoQ7
• CoQ4
• CoQ5
• CoQ8A and CoQ8B
• CoQ9

Anti-aging effect of CoQ10 supplementation:

The number of studies on CoQ10 supplementation has risen during the past decade, along with positive anti-aging and longevity results. Indeed, CoQ 10 antioxidant properties increase mitochondrial function by combating ROS production and reduce oxidative damage.

The dose and time of CoQ10 supplementation vary within studies, but most of them agree that the positive effects are strongly associated with a long-term supplementation period. This information is related to the observation of CoQ10 lack of accumulation after the ending of the supplementation period. 

Some of the results associated with CoQ 10 supplementation are:

• The attenuation of oxidative damage of proteins in the lived of aged mice.
• Reduced oxidative stress on muscle mitochondria due to increased antioxidant enzyme activation and higher levels of ROS scavengers.
• In humans, the supplementation of 1200mg/day in patients undergoing hemodialysis reduced F2-isoprostane concentration. 

Maintaining mitochondrial health and function is paramount to prevent aging-associated diseases. Mitochondrial energy production will inevitably lead to the formation of ROS. Therefore, Coenzyme Q10 supplementation has proven to delay aging reactions, protecting the mitochondria against ROS and preventing cardiometabolic diseases. – Ana Paola Rodríguez Arciniega, MS

References:

Díaz-Casado, M Elena et al. “The Paradox of Coenzyme Q10 in Aging.” Nutrients vol. 11,9 2221. 14 Sep. 2019, doi:10.3390/nu11092221

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