Mitochondrial cardiomyopathy and coenzyme Q₁₀

Heart mitochondrial oxidative phosphorylation function and coenzyme Q₁₀ (CoQ₁₀) concentration in clinical and experimental cardiomyopathies are presented. In endomyocardial biopsies (EMB) of patients with cardiomyopathy of unknown etiology (CPUE), decreased mitochondrial respiration, ATP production and CoQ₁₀ concentrations have been found. A positive relationship between the degree of rejection, decreased CoQ₁₀ and OXPHOS function in EMB of transplanted hearts has been documented. In experimental medicine circadian and circasemidian rhythms of heart mitochondrial "CoQ₁₀- CLOCK" have been found and different parameters of the cascade of oxidative phosphorylation between control and diabetic rats' hearts were estimated. Mapping changes in heart CoQ₁₀ and ATP production along the 24-hour scale can help obtain a better understanding of triggers of acute heart attacks. A novel CoQ binding site in Voltage- Dependent Anion Channel (VDAC) of outer mitochondrial membrane is proposed [4]. CoQ₁₀ targeted therapy of damaged mitochondria could be in the site of porin (VDAC) of the outer mitochondrial membrane where exogenous CoQ₁₀ passes through this channel into mitochondria. Based on current knowledge, target of mitochondrial respiratory chain supplementary therapy with CoQ₁₀ in cardiomyopathies is warranted.