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jws-hep.21313.fig1.TIF97K Mitochondrial respiratory chain (MRC).Mitochondrial -oxidation of fatty acids leads to the formation of short-chain acyl-CoA and acetyl-CoA and the conversion of oxidized nicotinamide adenine dinucleotide (NAD + ) and flavin adenine dinucleotide (FAD) into NADH and FADH 2 . MRC reoxidizes NADH and FADH 2into NAD +and FAD, respectively. Electrons from NADH and FADH 2are transferred to this enzymatic chain, where they are finally combined with oxygen and protons to form water. This transfer of electrons along the MRC is coupled with the exit of protons from the mitochondrial matrix into the intermembrane space creating an electrochemical potential across the inner membrane. Protons return to the matrix through adenosine triphosphate synthase, causing the conversion of adenosine diphosphate (ADP) into adenosine triphosphate (ATP). In normal conditions, a small fraction of electrons flowing along the MRC reacts with oxygen to form ROS. Impaired MRC function results in an increased ROS formation. NADH DH, NADH dehydrogenase. Succ.-DH, succinate dehydrogenase. Cyt. b-c 1 , cytochrome b-c 1 . Cyt. c, cytochrome c. Cyt. a-a 3oxidase, cytochrome oxidase complex. ROS, reactive oxygen species. UQ, ubiquinone.

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