TY - CHAP M1 - Book, Section TI - Mitochondrial Fatty Acid Oxidation Defects 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. PY - 2017 T2 - Pediatric Endocrinology and Inborn Errors of Metabolism, 2e 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. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/10/10 UR - accesspediatrics.mhmedical.com/content.aspx?aid=1140315409 ER -