RT Book, Section A1 Vockley, Jerry A1 Longo, Nicola A1 Andresen, Brage S. A1 Bennett, Michael J. A2 Sarafoglou, Kyriakie A2 Hoffmann, Georg F. A2 Roth, Karl S. SR Print(0) ID 1140315409 T1 Mitochondrial Fatty Acid Oxidation Defects T2 Pediatric Endocrinology and Inborn Errors of Metabolism, 2e YR 2017 FD 2017 PB McGraw-Hill Education PP New York, NY SN 9780071773140 LK accesspediatrics.mhmedical.com/content.aspx?aid=1140315409 RD 2024/10/10 AB Mitochondria can utilize carbohydrate, protein, or fat as a source of energy. Pyruvate, derived from glucose or amino acids, is transported through the mitochondrial inner membrane using a specific transporter,1 decarboxylated to acetyl-coenzyme A (CoA), and enters the citric acid cycle producing reducing equivalents in the form of 1,5-dihydroflavin adenine dinucleotide (FADH2) and nicotinamide adenine dinucleotide (NADH). These reducing equivalents are transported down the electron transport chain of inner mitochondrial membrane complexes, ultimately generating adenosine triphosphate (ATP) and consuming oxygen. Similarly, activated fatty acids (acyl-CoAs) entering the fatty acid β-oxidation (FAO) spiral generate reducing equivalents in the form of NADH and FADH2, which also pass down the oxidative phosphorylation respiratory chain complexes to generate ATP.