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Mitochondrial carnitine/acylcarnitine carrier protein Mitochondrial carnitine/acylcarnitine carrier protein Carnitine O- palmitoyltransferase 2, mitochondrial Long-chain- fatty-acid-- CoA ligase 1 Carnitine O- palmitoyltransferase 2, mitochondrial Enoyl-CoA hydratase, mitochondrial Trifunctional enzyme subunit alpha, mitochondrial Acetyl-CoA acetyltransferase, cytosolic Hydroxyacyl- coenzyme A dehydrogenase, mitochondrial Trifunctional enzyme subunit alpha, mitochondrial Acetyl-CoA acetyltransferase, cytosolic Trifunctional enzyme subunit alpha, mitochondrial Acetyl-CoA acetyltransferase, cytosolic Enoyl-CoA hydratase, mitochondrial Trifunctional enzyme subunit alpha, mitochondrial Acetyl-CoA acetyltransferase, cytosolic Hydroxyacyl- coenzyme A dehydrogenase, mitochondrial Acetyl-CoA acetyltransferase, mitochondrial Acetyl-CoA acetyltransferase, mitochondrial Trifunctional enzyme subunit alpha, mitochondrial Acetyl-CoA acetyltransferase, cytosolic Trifunctional enzyme subunit alpha, mitochondrial Acetyl-CoA acetyltransferase, cytosolic Enoyl-CoA hydratase, mitochondrial Trifunctional enzyme subunit alpha, mitochondrial Acetyl-CoA acetyltransferase, cytosolic Hydroxyacyl- coenzyme A dehydrogenase, mitochondrial Trifunctional enzyme subunit alpha, mitochondrial Acetyl-CoA acetyltransferase, cytosolic Acetyl-CoA acetyltransferase, mitochondrial Trifunctional enzyme subunit alpha, mitochondrial Acetyl-CoA acetyltransferase, cytosolic 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 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 Slc25a20 Slc25a20 Cpt2 Acsl1 Cpt2 Echs1 Hadha Acat2 Hadh Hadha Acat2 Hadha Acat2 Echs1 Hadha Acat2 Hadh Acat1 Acat1 Hadha Acat2 Hadha Acat2 Echs1 Hadha Acat2 Hadh Hadha Acat2 Acat1 Hadha Acat2 Acadl Acadl Acadl 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