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Mitochondrial basic amino acids transporter Acyl-coenzyme A synthetase ACSM1, 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 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 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 Medium-chain specific acyl-CoA dehydrogenase, mitochondrial Medium-chain specific acyl-CoA dehydrogenase, mitochondrial Medium-chain specific acyl-CoA dehydrogenase, mitochondrial Dodecanoic acid Lauroyl-CoA Acetyl-CoA Acetyl-CoA Acetyl-CoA Hexanoyl-CoA Dodecanoic acid ATP CoA AMP PPi 3-Hydroxydodecanoyl-CoA (2E)-Dodecenoyl-CoA H2O H2O NAD 3-Oxododecanoyl-CoA NADH H+ NAD NADH H+ Decanoyl-CoA CoA CoA 3-Hydroxydecanoyl-CoA (2E)-Decenoyl-CoA H2O H2O NAD 3-Oxodecanoyl-CoA H+ NADH NAD H+ NAD Octanoyl-CoA CoA CoA 3-Hydroxyoctanoyl-CoA (2E)-Octenoyl-CoA H2O H2O NAD 3-Oxooctanoyl-CoA H+ NADH NAD H+ NADH 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 Mitochondrial Beta-Oxidation of Long Chain Saturated Fatty Acids Citric Acid Cycle Citric Acid Cycle Citric Acid Cycle Mitochondrial Beta-Oxidation of Short Chain Saturated Fatty Acids Mitochondrial Inner Membrane Mitochondrial Outer Membrane Mitochondrial Inner Membrane Mitochondrial Matrix Mitochondria Extracellular Space Intracellular Space
Mitochondria SLC25A29 ACSM1 ECHS1 HADHA HADHB HADH HADHA HADHB ACAA2 HADHA HADHB ECHS1 HADHA HADHB HADH HADHA HADHB ACAA2 HADHA HADHB ECHS1 HADHA HADHB HADH HADHA HADHB ACAA2 HADHA HADHB ACADM ACADM ACADM Dodecanoic acid Lauroyl-CoA Acetyl-CoA Acetyl-CoA Acetyl-CoA Hexanoyl-CoA Dodecanoic acid Adenosine triphosphate Coenzyme A Adenosine monophosphate Pyrophosphate 3- Hydroxydodecanoyl- CoA (2E)- Dodecenoyl- CoA Water Water NAD 3- Oxododecanoyl- CoA NADH Hydrogen Ion NAD NADH Hydrogen Ion Decanoyl-CoA Coenzyme A Coenzyme A 3- Hydroxydecanoyl- CoA (2E)- Decenoyl-CoA Water Water NAD 3- Oxodecanoyl- CoA Hydrogen Ion NADH NAD Hydrogen Ion NADH Octanoyl-CoA Coenzyme A Coenzyme A 3- Hydroxyoctanoyl- CoA (2E)- Octenoyl-CoA Water Water NAD 3- Oxooctanoyl- CoA Hydrogen Ion NADH NAD Hydrogen Ion NADH 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 Mitochondrial Beta-Oxidation of Long Chain Saturated Fatty Acids Citric Acid Cycle Citric Acid Cycle Citric Acid Cycle Mitochondrial Beta-Oxidation of Short Chain Saturated Fatty Acids
SLC25A29 ACSM1 ECHS1 HADHA HADHB HADH HADHA HADHB ACAA2 HADHA HADHB ECHS1 HADHA HADHB HADH HADHA HADHB ACAA2 HADHA HADHB ECHS1 HADHA HADHB HADH HADHA HADHB ACAA2 HADHA HADHB ACADM ACADM ACADM 12:0 COA12:0 Ac-CoA Ac-CoA Ac-CoA COA6:0 12:0 ATP CoA AMP PPi S3HDCoA DDC-CoA H2O H2O NAD 3ODDCoA NADH H+ NAD NADH H+ Decanoy CoA CoA SHD-CoA 2DCoA H2O H2O NAD 3ODCCoA H+ NADH NAD H+ NAD COA8:0 CoA CoA S-HOCoA 2OCoA H2O H2O NAD 3OOACoA H+ NADH NAD H+ NADH CoA CoA Mg2+ FAD FAD FAD ETF RETF ETF RETF ETF RETF PW000167 PW000022 PW000173 PW000005 PW000005 PW000005 PW000171 Mitochondrial Inner Membrane Mitochondrial Outer Membrane Mitochondrial Inner Membrane Mitochondrial Matrix Mitochondria Extracellular Space Intracellular Space
Mitochondria SLC25A29 ACSM1 ECHS1 HADHA HADHB HADH HADHA HADHB ACAA2 HADHA HADHB ECHS1 HADHA HADHB HADH HADHA HADHB ACAA2 HADHA HADHB ECHS1 HADHA HADHB HADH HADHA HADHB ACAA2 HADHA HADHB ACADM ACADM ACADM 12:0 COA12:0 Ac-CoA Ac-CoA Ac-CoA COA6:0 12:0 ATP CoA AMP Ppi S3HDCoA DDC-CoA H2O H2O NAD 3ODDCoA NADH H+ NAD NADH H+ Decanoy CoA CoA SHD-CoA 2DCoA H2O H2O NAD 3ODCCoA H+ NADH NAD H+ NADH COA8:0 CoA CoA S-HOCoA 2OCoA H2O H2O NAD 3OOACoA H+ NADH NAD H+ NADH CoA CoA ETF RETF ETF RETF ETF RETF PW000167 PW000022 PW000173 PW000005 PW000005 PW000005 PW000171