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Mitochondrial metabolic pathways

Selected metabolic pathways in mitochondria. The spirals represent the spiral reactions of the beta-oxidation pathway, resulting in the liberation of acetyl-coenzyme A (CoA) and the reduction of flavoprotein. Abbreviations: ADP, adenosine diphosphate; ATP, adenosine triphosphate; ANT, adenine nucleotide translocator; CACT, carnitine-acylcarnitine translocase; CoQ, coenzyme Q; CPT, carnitine palmitoyltransferase; DIC, dicarboxylate carrier; ETF, electron-transfer flavoprotein; ETFDH, electron-transfer dehydrogenase; FAD, flavin adenine dinucleotide; FADH2, reduced FAD; NADH, reduced nicotinamide adenine dinucleotide; PDH, pyruvate dehydrogenase complex; TCA, tricarboxylic acid; I, complex I; II, complex II; III, complex III; IV, complex IV; V, complex V (ATP synthase). (Contributed by Dr. Salvatore DiMauro.)

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