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Mitochondrial carnitine/acylcarnitine carrier protein Mitochondrial carnitine/acylcarnitine carrier protein Carnitine O- palmitoyltransferase 1, liver isoform Long-chain- fatty-acid-- CoA ligase 1 Carnitine O- palmitoyltransferase 2, mitochondrial Enoyl-CoA hydratase, mitochondrial Trifunctional enzyme subunit alpha, mitochondrial Trifunctional enzyme subunit beta, mitochondrial Hydroxyacyl- coenzyme A dehydrogenase, mitochondrial Trifunctional enzyme subunit alpha, mitochondrial Trifunctional enzyme subunit beta, mitochondrial Trifunctional enzyme subunit alpha, mitochondrial Trifunctional enzyme subunit beta, mitochondrial Enoyl-CoA hydratase, mitochondrial Trifunctional enzyme subunit alpha, mitochondrial Trifunctional enzyme subunit beta, mitochondrial Hydroxyacyl- coenzyme A dehydrogenase, mitochondrial 3-ketoacyl-CoA thiolase, mitochondrial 3-ketoacyl-CoA thiolase, mitochondrial Trifunctional enzyme subunit alpha, mitochondrial Trifunctional enzyme subunit beta, mitochondrial Trifunctional enzyme subunit alpha, mitochondrial Trifunctional enzyme subunit beta, mitochondrial Enoyl-CoA hydratase, mitochondrial Trifunctional enzyme subunit alpha, mitochondrial Trifunctional enzyme subunit beta, mitochondrial Hydroxyacyl- coenzyme A dehydrogenase, mitochondrial Trifunctional enzyme subunit alpha, mitochondrial Trifunctional enzyme subunit beta, mitochondrial 3-ketoacyl-CoA thiolase, mitochondrial Trifunctional enzyme subunit alpha, mitochondrial Trifunctional enzyme subunit beta, mitochondrial Long-chain specific acyl-CoA dehydrogenase, mitochondrial Long-chain specific acyl-CoA dehydrogenase, mitochondrial Long-chain specific acyl-CoA dehydrogenase, mitochondrial Stearic acid Acetyl-CoA Acetyl-CoA Acetyl-CoA Lauroyl-CoA L-Carnitine L-Carnitine Stearoylcarnitine Stearoylcarnitine Stearoyl-CoA CoA CoA ATP PPi AMP Stearoyl-CoA CoA (2E)-Octadecenoyl-CoA H2O (S)-Hydroxyoctadecanoyl-CoA H2O NAD 3-oxooctadecanoyl-CoA NADH H+ NAD NADH H+ CoA Palmitoyl-CoA 3-Hydroxyhexadecanoyl-CoA (2E)-Hexadecenoyl-CoA H2O H2O NAD 3-oxohexadecanoyl-CoA NADH H+ Tetradecanoyl-CoA CoA CoA CoA NAD NADH H+ 3-Hydroxytetradecanoyl-CoA (2E)-Tetradecenoyl-CoA H2O H2O NAD 3-oxotetradecanoyl-CoA NADH H+ NAD NADH H+ CoA CoA electron- transfer flavoprotein Reduced electron- transfer flavoprotein electron- transfer flavoprotein Reduced electron- transfer flavoprotein electron- transfer flavoprotein Reduced electron- transfer flavoprotein Fatty Acid Biosynthesis Fatty Acid Elongation in Mitochondria Citric Acid Cycle Citric Acid Cycle Citric Acid Cycle Mitochondrial Beta-Oxidation of Medium Chain Saturated Fatty Acids Magnesium FAD FAD FAD Extracellular Space Intracellular Space Mitochondrial Outer Membrane Mitochondrial Inner Membrane Mitochondrial Matrix Mitochondria
Mitochondria SLC25A20 SLC25A20 CPT1A ACSL1 CPT2 ECHS1 HADHA