Human Endogenous Metabolite
Coenzyme Q9 (5 mg/kg; p.o.; once a day for 4 weeks) reduces myocardial ischemia/reperfusion injury. CoQ 10 and CoQ 9 are components of themitochondrial respiratory chain in mammals and can regulate some mitochondrial proteins/functions. Soybean, corn, and rapeseed oils are very rich sources of CoQ 10 , whereas CoQ 9 has been found in high concentrations in corn oil. The lack of a functional CoQ 9 protein in homozygous CoQ 9 mutant (CoQ 9 (X/X)) mice causes a severe reduction in the CoQ 7 protein and a widespread CoQ deficiency and accumulation of demethoxyubiquinone. The deficit in CoQ induces a brain-specific impairment of mitochondrial bioenergetics performance, a reduction in respiratory control ratio, ATP levels and ATP/ADP ratio and specific loss of respiratory complex I. These effects lead to neuronal death and demyelinization with severe vacuolization and astrogliosis in the brain of CoQ 9 (X/X) mice that consequently die between 3 and 6 months of age.