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Here, CoQ 10 is kept in equilibrium between its redox forms outside the mitochondria by the actions of three enzymes: nicotinamide adenine dinucleotide reduced/nicotinamide adenine dinucleotide phosphate (NADH/NADPH) reduced oxidoreductase, NADH cytochrome b 5 reductase, and NADPH-coenzyme Q reductase (Figure 1) [6,7,8]

Defects in complex I are among the most common causes of mitochondrial diseases, contributing to disorders such as Leigh syndrome, mitochondrial encephalomyopathy and Parkinsons disease by disrupting ATP production and elevating oxidative stress 21,71,72,73

DOI: 10.1038/ejcn.2010.136 72