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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 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 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 Palmitoyl-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 Hydrogen Ion 3- Hydroxytetradecanoyl- CoA (2E)- Tetradecenoyl- CoA Water Water NAD 3- oxotetradecanoyl- CoA NADH Hydrogen Ion NAD NADH Hydrogen Ion Coenzyme A Coenzyme A 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
SLC25A20 SLC25A20 CPT1A ACSL1 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 ACADL ACADL ACADL 18:0 Ac-CoA Ac-CoA Ac-CoA COA12:0 L-Carnt L-Carnt Stearoy Stearoy COA18:0 CoA CoA ATP PPi AMP COA18:0 CoA tO2eCoA H2O SHCoA H2O NAD 3OOACoA NADH H+ NAD NADH H+ CoA COA16:0 BHC (E)-C16 H2O H2O NAD 3OHDCoA NADH H+ COA14:0 CoA CoA CoA NAD NADH H+ S3HTCoA 2TCoA H2O H2O NAD 3OTDCoA NADH H+ NAD NADH H+ CoA CoA ETF RETF ETF RETF ETF RETF PW000167 PW000022 PW000005 PW000005 PW000005 PW000172 Mg2+ FAD FAD FAD Extracellular Space Intracellular Space Mitochondrial Outer Membrane Mitochondrial Inner Membrane Mitochondrial Matrix Mitochondria
Mitochondria SLC25A20 SLC25A20 CPT1A ACSL1 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 ACADL ACADL ACADL 18:0 Ac-CoA Ac-CoA Ac-CoA COA12:0 L-Carnt L-Carnt Stearoy Stearoy COA18:0 CoA CoA ATP Ppi AMP COA18:0 CoA tO2eCoA H2O SHCoA H2O NAD 3OOACoA NADH H+ NAD NADH H+ CoA COA16:0 BHC (E)-C16 H2O H2O NAD 3OHDCoA NADH H+ COA14:0 CoA CoA CoA NAD NADH H+ S3HTCoA 2TCoA H2O H2O NAD 3OTDCoA NADH H+ NAD NADH H+ CoA CoA ETF RETF ETF RETF ETF RETF PW000167 PW000022 PW000005 PW000005 PW000005 PW000172