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Carnitine O- palmitoyltransferase 2, mitochondrial Enoyl-CoA hydratase, mitochondrial Trifunctional enzyme subunit alpha, mitochondrial Trifunctional enzyme subunit beta, mitochondrial 3-ketoacyl-CoA thiolase, mitochondrial Carnitine O- palmitoyltransferase 1, liver isoform Long-chain- fatty-acid-- CoA ligase 1 Enoyl-CoA hydratase, 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 3-ketoacyl-CoA thiolase, mitochondrial Enoyl-CoA hydratase, mitochondrial Trifunctional enzyme subunit alpha, mitochondrial Trifunctional enzyme subunit beta, mitochondrial 3-ketoacyl-CoA thiolase, mitochondrial Enoyl-CoA hydratase, mitochondrial Trifunctional enzyme subunit alpha, mitochondrial Trifunctional enzyme subunit beta, mitochondrial 3-ketoacyl-CoA thiolase, mitochondrial Long-chain specific acyl-CoA dehydrogenase, mitochondrial Medium-chain specific acyl-CoA dehydrogenase, mitochondrial Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial Short-chain specific acyl-CoA dehydrogenase, mitochondrial Enoyl-CoA hydratase, mitochondrial Trifunctional enzyme subunit alpha, mitochondrial Trifunctional enzyme subunit beta, mitochondrial 3-ketoacyl-CoA thiolase, mitochondrial Long-chain specific acyl-CoA dehydrogenase, mitochondrial Medium-chain specific acyl-CoA dehydrogenase, mitochondrial Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial Enoyl-CoA hydratase, mitochondrial Acetyl-CoA acetyltransferase, mitochondrial Glutaryl-CoA dehydrogenase, mitochondrial Very long-chain specific acyl-CoA dehydrogenase, mitochondrial Long-chain specific acyl-CoA dehydrogenase, mitochondrial Medium-chain specific acyl-CoA dehydrogenase, mitochondrial Long-chain specific acyl-CoA dehydrogenase, mitochondrial Medium-chain specific acyl-CoA dehydrogenase, mitochondrial Long-chain specific acyl-CoA dehydrogenase, mitochondrial Medium-chain specific acyl-CoA dehydrogenase, mitochondrial Enoyl-CoA hydratase, mitochondrial Long-chain specific acyl-CoA dehydrogenase, mitochondrial Medium-chain specific acyl-CoA dehydrogenase, mitochondrial Long-chain specific acyl-CoA dehydrogenase, mitochondrial Medium-chain specific acyl-CoA dehydrogenase, mitochondrial Trifunctional enzyme subunit alpha, mitochondrial Trifunctional enzyme subunit beta, mitochondrial Palmitic acid L-Palmitoylcarnitine L-Palmitoylcarnitine Palmitoyl-CoA L-Carnitine CoA 3-Hydroxyhexadecanoyl-CoA (2E)-Hexadecenoyl-CoA H2O NAD 3-oxohexadecanoyl-CoA NADH H+ Tetradecanoyl-CoA Acetyl-CoA CoA Palmitoyl-CoA L-Carnitine CoA ATP CoA AMP PPi 3-Hydroxytetradecanoyl-CoA (2E)-Tetradecenoyl-CoA H2O NAD 3-oxotetradecanoyl-CoA NADH H+ Lauroyl-CoA Acetyl-CoA CoA 3-Hydroxydodecanoyl-CoA NAD 3-oxododecanoyl-CoA NADH H+ Decanoyl-CoA Acetyl-CoA CoA 3-Hydroxydecanoyl-CoA (2E)-Decenoyl-CoA H2O NAD 3-oxodecanoyl-CoA H+ NADH Octanoyl-CoA Acetyl-CoA CoA 3-hydroxyoctanoyl-CoA (2E)-Octenoyl-CoA H2O NAD 3-oxooctanoyl-CoA H+ NADH Acetyl-CoA Hexanoyl-CoA CoA (2E)-Hexenoyl-CoA 3-Hydroxyhexanoyl-CoA H2O NAD 3-oxohexanoyl-CoA NADH H+ Acetyl-CoA Butyryl-CoA CoA Crotonoyl-CoA 3-Hydroxybutyryl-CoA H2O Acetyl-CoA Acetoacetyl-CoA CoA Glutaryl-CoA CO2 NAD NADH H+ (2E)-Tetradecenoyl-CoA H2O Magnesium FAD FAD FAD FAD FAD electron- transfer flavoprotein Reduced electron- transfer flavoprotein electron- transfer flavoprotein Reduced electron- transfer flavoprotein electron- transfer flavoprotein Reduced electron- transfer flavoprotein electron- transfer flavoprotein Reduced electron- transfer flavoprotein electron- transfer flavoprotein Reduced electron- transfer flavoprotein electron- transfer flavoprotein Reduced electron- transfer flavoprotein Fatty Acid Elongation in Mitochondria Glycerolipid Metabolism Phospholipid Biosynthesis FAD FAD FAD FAD FAD FAD FAD FAD FAD FAD FAD FAD FAD FAD Mitochondrial Matrix Inner Mitochondria Membrane Mitochondrial Intermembrane Space Outer Mitochondria Membrane Cytosol Extracellular Space Severe multisystemic diseases characterized by liver failure with hypoketotic hypoglycemia, cardiomyopathy, seizures, and early death. The adult-onset myopathic form is characterized by exercise-induced muscle pain and weakness, sometimes associated with myoglobinuria.
Mitochondria CPT2 ECHS1 HADHA HADHB ACAA2 CPT1A ACSL1 ECHS1 HADHA HADHB ACAA2 HADHA HADHB ACAA2 ECHS1 HADHA HADHB ACAA2 ECHS1 HADHA HADHB ACAA2 ACADL ACADM ACADSB ACADS ECHS1 HADHA HADHB ACAA2 ACADL ACADM ACADSB ECHS1 ACAT1 GCDH ACADVL ACADL ACADM ACADL ACADM ACADL ACADM ECHS1 ACADL ACADM ACADL ACADM HADHA HADHB Palmitic acid L- Palmitoylcarnitine L- Palmitoylcarnitine Palmitoyl-CoA L-Carnitine Coenzyme A 3- Hydroxyhexadecanoyl- CoA (2E)- Hexadecenoyl- CoA Water NAD 3- oxohexadecanoyl- CoA NADH Hydrogen Ion Tetradecanoyl- CoA Acetyl-CoA Coenzyme A Palmitoyl-CoA L-Carnitine Coenzyme A Adenosine triphosphate Coenzyme A Adenosine monophosphate Pyrophosphate 3- Hydroxytetradecanoyl- CoA (2E)- Tetradecenoyl- CoA Water NAD 3- oxotetradecanoyl- CoA NADH Hydrogen Ion Lauroyl-CoA Acetyl-CoA Coenzyme A 3- Hydroxydodecanoyl- CoA NAD 3- oxododecanoyl- CoA NADH Hydrogen Ion Decanoyl-CoA Acetyl-CoA Coenzyme A 3- Hydroxydecanoyl- CoA (2E)- Decenoyl-CoA Water NAD 3- oxodecanoyl- CoA Hydrogen Ion NADH Octanoyl-CoA Acetyl-CoA Coenzyme A 3- hydroxyoctanoyl- CoA (2E)- Octenoyl-CoA Water NAD 3- oxooctanoyl- CoA Hydrogen Ion NADH Acetyl-CoA Hexanoyl-CoA Coenzyme A (2E)- Hexenoyl-CoA 3- Hydroxyhexanoyl- CoA Water NAD 3- oxohexanoyl- CoA NADH Hydrogen Ion Acetyl-CoA Butyryl-CoA Coenzyme A Crotonoyl-CoA 3- Hydroxybutyryl- CoA Water Acetyl-CoA Acetoacetyl-CoA Coenzyme A Glutaryl-CoA Carbon dioxide NAD NADH Hydrogen Ion (2E)- Tetradecenoyl- CoA Water electron- transfer flavoprotein Reduced electron- transfer flavoprotein electron- transfer flavoprotein Reduced electron- transfer flavoprotein electron- transfer flavoprotein Reduced electron- transfer flavoprotein electron- transfer flavoprotein Reduced electron- transfer flavoprotein electron- transfer flavoprotein Reduced electron- transfer flavoprotein electron- transfer flavoprotein Reduced electron- transfer flavoprotein Fatty Acid Elongation in Mitochondria Glycerolipid Metabolism Phospholipid Biosynthesis
CPT2 ECHS1 HADHA HADHB ACAA2 CPT1A ACSL1 ECHS1 HADHA HADHB ACAA2 HADHA HADHB ACAA2 ECHS1 HADHA HADHB ACAA2 ECHS1 HADHA HADHB ACAA2 ACADL ACADM ACADSB ACADS ECHS1 HADHA HADHB ACAA2 ACADL ACADM ACADSB ECHS1 ACAT1 GCDH ACADVL ACADL ACADM ACADL ACADM ACADL ACADM ECHS1 ACADL ACADM ACADL ACADM HADHA HADHB 16:0 L-Plmtc L-Plmtc COA16:0 L-Carnt CoA BHC (E)-C16 H2O NAD 3OHDCoA NADH H+ COA14:0 Ac-CoA CoA COA16:0 L-Carnt CoA ATP CoA AMP PPi S3HTCoA 2TCoA H2O NAD 3OTDCoA NADH H+ COA12:0 Ac-CoA CoA S3HDCoA NAD 3ODDCoA NADH H+ Decanoy Ac-CoA CoA SHD-CoA 2DCoA H2O NAD 3ODCCoA H+ NADH COA8:0 Ac-CoA CoA S-HOCoA 2OCoA H2O NAD 3OOACoA H+ NADH Ac-CoA COA6:0 CoA T2HCoA S-HHxCA H2O NAD 3OHOCoA NADH H+ Ac-CoA Btyl-CA CoA CrtylCA 3HB-CoA H2O Ac-CoA ActaCoA CoA GlutCoA CO2 NAD NADH H+ 2TCoA H2O Mg2+ FAD FAD FAD FAD FAD ETF RETF ETF RETF ETF RETF ETF RETF ETF RETF ETF RETF PW000022 PW000144 PW000048 FAD FAD FAD FAD FAD FAD FAD FAD FAD FAD FAD FAD FAD FAD Mitochondrial Matrix Inner Mitochondria Membrane Mitochondrial Intermembrane Space Outer Mitochondria Membrane Cytosol Extracellular Space Severe multisystemic diseases characterized by liver failure with hypoketotic hypoglycemia, cardiomyopathy, seizures, and early death. The adult-onset myopathic form is characterized by exercise-induced muscle pain and weakness, sometimes associated with myoglobinuria.
Mitochondria CPT2 ECHS1 HADHA HADHB ACAA2 CPT1A ACSL1 ECHS1 HADHA HADHB ACAA2 HADHA HADHB ACAA2 ECHS1 HADHA HADHB ACAA2 ECHS1 HADHA HADHB ACAA2 ACADL ACADM ACADSB ACADS ECHS1 HADHA HADHB ACAA2 ACADL ACADM ACADSB ECHS1 ACAT1 GCDH ACADVL ACADL ACADM ACADL ACADM ACADL ACADM ECHS1 ACADL ACADM ACADL ACADM HADHA HADHB 16:0 L-Plmtc L-Plmtc COA16:0 L-Carnt CoA BHC (E)-C16 H2O NAD 3OHDCoA NADH H+ COA14:0 Ac-CoA CoA COA16:0 L-Carnt CoA ATP CoA AMP Ppi S3HTCoA 2TCoA H2O NAD 3OTDCoA NADH H+ COA12:0 Ac-CoA CoA S3HDCoA NAD 3ODDCoA NADH H+ Decanoy Ac-CoA CoA SHD-CoA 2DCoA H2O NAD 3ODCCoA H+ NADH COA8:0 Ac-CoA CoA S-HOCoA 2OCoA H2O NAD 3OOACoA H+ NADH Ac-CoA COA6:0 CoA T2HCoA S-HHxCA H2O NAD 3OHOCoA NADH H+ Ac-CoA Btyl-CA CoA CrtylCA 3HB-CoA H2O Ac-CoA ActaCoA CoA GlutCoA CO2 NAD NADH H+ 2TCoA H2O ETF RETF ETF RETF ETF RETF ETF RETF ETF RETF ETF RETF PW000022 PW000144 PW000048