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Mitochondrial basic amino acids transporter Mitochondrial basic amino acids transporter Carnitine O- palmitoyltransferase 1, muscle isoform Long-chain- fatty-acid-- CoA ligase ACSBG1 Carnitine O- palmitoyltransferase 2, mitochondrial Enoyl-CoA hydratase, mitochondrial HADHA protein Trifunctional enzyme subunit beta, mitochondrial Hydroxyacyl-CoA dehydrogenase HADHA protein Trifunctional enzyme subunit beta, mitochondrial HADHA protein Trifunctional enzyme subunit beta, mitochondrial Enoyl-CoA hydratase, mitochondrial HADHA protein Trifunctional enzyme subunit beta, mitochondrial Hydroxyacyl-CoA dehydrogenase 3-ketoacyl-CoA thiolase, mitochondrial 3-ketoacyl-CoA thiolase, mitochondrial HADHA protein Trifunctional enzyme subunit beta, mitochondrial HADHA protein Trifunctional enzyme subunit beta, mitochondrial Enoyl-CoA hydratase, mitochondrial HADHA protein Trifunctional enzyme subunit beta, mitochondrial Hydroxyacyl-CoA dehydrogenase HADHA protein Trifunctional enzyme subunit beta, mitochondrial 3-ketoacyl-CoA thiolase, mitochondrial HADHA protein Trifunctional enzyme subunit beta, mitochondrial Isovaleryl-CoA dehydrogenase, mitochondrial Isovaleryl-CoA dehydrogenase, mitochondrial Isovaleryl-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 Palmityl-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 Magnesium FAD FAD FAD 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 Extracellular Space Intracellular Space Mitochondrial Outer Membrane Mitochondrial Inner Membrane Mitochondrial Matrix Mitochondria
Mitochondria SLC25A29 SLC25A29 CPT1B ACSBG1 CPT2 ECHS1 HADHA HADHB HADH HADHA HADHB HADHA HADHB ECHS1 HADHA HADHB HADH ACAA2 ACAA2 HADHA HADHB HADHA HADHB ECHS1 HADHA HADHB HADH HADHA HADHB ACAA2 HADHA HADHB IVD IVD IVD Stearic acid Acetyl-CoA Acetyl-CoA Acetyl-CoA Lauroyl-CoA L-Carnitine L-Carnitine Stearoylcarnitine Stearoylcarnitine Stearoyl-CoA Coenzyme A Coenzyme A Adenosine triphosphate Pyrophosphate Adenosine monophosphate Stearoyl-CoA Coenzyme A (2E)- Octadecenoyl- CoA Water (S)- Hydroxyoctadecanoyl- CoA Water NAD 3- Oxooctadecanoyl- CoA NADH Hydrogen Ion NAD NADH Hydrogen Ion Coenzyme A Palmityl-CoA 3- Hydroxyhexadecanoyl- CoA (2E)- Hexadecenoyl- CoA Water Water NAD 3- Oxohexadecanoyl- CoA NADH Hydrogen Ion Tetradecanoyl- CoA Coenzyme A Coenzyme A Coenzyme A NAD NADH </