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Solute carrier family 13 member 3 Solute carrier family 22 member 7 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 Medium-chain specific acyl-CoA dehydrogenase, mitochondrial Methylglutaconyl- CoA hydratase, mitochondrial Succinate-- hydroxymethylglutarate CoA-transferase Unknown Trifunctional enzyme subunit alpha, mitochondrial Trifunctional enzyme subunit beta, mitochondrial Palmitic acid L-Palmitoylcarnitine L-Palmitoylcarnitine 3-Hydroxyglutaric acid Glutaric acid Glutaric acid 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 H+ Glutaconyl-CoA H2O 3-Hydroxy-3-methylglutaryl-CoA Succinate Succinyl-CoA ADP Pi ATP CoA Magnesium FAD FAD FAD FAD FAD 3-Hydroxyglutaric acid 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 Oxidized flavoprotein Reduced flavoprotein Solute carrier family 13 member 3 Solute carrier family 22 member 7 Fatty Acid Elongation in Mitochondria Glycerolipid Metabolism Phospholipid Biosynthesis FAD 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 Symptoms include encephalopathy, grimacing, dystonia, metabolic acidosis, and hygroma.
Mitochondria SLC13A3 SLC22A7 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 ACADM AUH SUGCT Unknown HADHA HADHB Palmitic acid L- Palmitoylcarnitine L- Palmitoylcarnitine 3- Hydroxyglutaric acid Glutaric acid Glutaric acid 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 Hydrogen Ion Glutaconyl-CoA Water 3-Hydroxy-3- methylglutaryl- CoA Succinate Succinyl-CoA Adenosine diphosphate Phosphate Adenosine triphosphate Coenzyme A 3- Hydroxyglutaric acid 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 Oxidized flavoprotein Reduced flavoprotein SLC13A3 SLC22A7 Fatty Acid Elongation in Mitochondria Glycerolipid Metabolism Phospholipid Biosynthesis
SLC13A3 SLC22A7 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 ACADM AUH SUGCT HADHA HADHB 16:0 L-Plmtc L-Plmtc 3H Glutara Glutara 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 H+ GluCCoA H2O HMG-CoA Succi Suc-CoA ADP Pi ATP CoA Mg2+ FAD FAD FAD FAD FAD 3H ETF RETF ETF RETF ETF RETF ETF RETF ETF RETF ETF RETF OF RF SLC13A3 SLC22A7 PW000022 PW000144 PW000048 FAD 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 Symptoms include encephalopathy, grimacing, dystonia, metabolic acidosis, and hygroma.
Mitochondria SLC13A3 SLC22A7 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 ACADM AUH SUGCT HADHA HADHB 16:0 L-Plmtc L-Plmtc 3H Glutara Glutara 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 H+ GluCCoA H2O HMG-CoA Succi Suc-CoA ADP Pi ATP CoA 3H ETF RETF ETF RETF ETF RETF ETF RETF ETF RETF ETF RETF OF RF SLC13A3 SLC22A7 PW000022 PW000144 PW000048