Аннотация
Cytokine Coenzyme Q10 (CoQ10) is an insoluble coenzyme that is a component of the electron transport chain and is involved in oxidative phosphorylation in mitochondria. By a decrease in CoQ10, the energy requirements of the cell are not fully covered: cell division slows down, and the processes of tissue renewal and regeneration are disrupted. In our work, we used a model of mice CoQ9 Knockin (R239X). In homozygous mutant mice, a strong decrease in CoQ7 protein and the accumulation of dimethoxyubiquinone are observed. An impairment of hydrogen sulfide metabolism was previously identified as a significant pathomechanism of primary Coenzyme Q deficiency. The experimental results showed that the disruption of sulfide and glutathione metabolism in Coq9R239X mice is directly dependent on CoQ levels. Therefore, changes in dietary sulfur-containing amino acids do not alter sulfide metabolism and the glutathione system in Coq9R239X mice. As our experiments have shown, an increase in total glutathione is observed only in symptomatic brain tissue. It can be assumed that this is due to oxidative stress in the symptomatic brain tissue, which is often found with mitochondrial dysfunction.