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Low affinity cationic amino acid transporter 2 Mitochondrial ornithine transporter 2 Unknown Solute carrier family 13 member 3 Alpha- aminoadipic semialdehyde synthase, mitochondrial Alpha- aminoadipic semialdehyde synthase, mitochondrial Alpha- aminoadipic semialdehyde dehydrogenase Kynurenine/alpha- aminoadipate aminotransferase, mitochondrial Enoyl-CoA hydratase, mitochondrial Hydroxyacyl- coenzyme A dehydrogenase, mitochondrial Acetyl-CoA acetyltransferase, mitochondrial Peroxisomal sarcosine oxidase Glutaryl-CoA dehydrogenase, mitochondrial Oxoglutarate dehydrogenase complex Medium-chain specific acyl-CoA dehydrogenase, mitochondrial Methylglutaconyl- CoA hydratase, mitochondrial Succinate-- hydroxymethylglutarate CoA-transferase Acetyl-CoA L-Lysine L-Lysine L-Lysine Allysine Allysine 3-Hydroxyglutaric acid 3-Hydroxyglutaric acid NADPH Oxoglutaric acid Saccharopine NADP H2O H2O NAD L-Glutamic acid NADH NAD H2O Aminoadipic acid NADH Oxoglutaric acid Oxoadipic acid L-Glutamic acid 3-Hydroxybutyryl-CoA Crotonoyl-CoA H2O NAD Acetoacetyl-CoA NADH CoA L-Pipecolic acid O2 (S)-2,3,4,5- Tetrahydropiperidine-2- carboxylate H2O2 Glutaryl-CoA CO2 CoA H2O H+ Glutaconyl-CoA H2O 3-Hydroxy-3-methylglutaryl-CoA Succinate Succinyl-CoA Pyridoxal 5'-phosphate Thiamine pyrophosphate Lipoamide FAD FAD Oxidized flavoprotein Reduced flavoprotein Solute carrier family 13 member 3 Citric Acid Cycle Biotin Metabolism FAD FAD Inner Mitochondria Membrane Outer Mitochondria Membrane Mitochondrial Intermembrane Space Peroxisome Mitochondria Extracellular Space Intracellular Space Liver Extracellular Space Mitochondrial matrix Liver Hepatic Cell Symptoms include encephalopathy, grimacing, dystonia, metabolic acidosis, and hygroma.
Mitochondria SLC7A2 SLC25A2 Unknown SLC13A3 AASS AASS ALDH7A1 AADAT ECHS1 HADH ACAT1 PIPOX GCDH DHTKD1 ACADM AUH SUGCT Acetyl-CoA L-Lysine L-Lysine L-Lysine Allysine Allysine 3- Hydroxyglutaric acid 3- Hydroxyglutaric acid NADPH Oxoglutaric acid Saccharopine NADP Water Water NAD L-Glutamic acid NADH NAD Water Aminoadipic acid NADH Oxoglutaric acid Oxoadipic acid L-Glutamic acid 3- Hydroxybutyryl- CoA Crotonoyl-CoA Water NAD Acetoacetyl-CoA NADH Coenzyme A L-Pipecolic acid Oxygen (S)-2,3,4,5- Tetrahydropiperidine- 2-carboxylate Hydrogen peroxide Glutaryl-CoA Carbon dioxide Coenzyme A Water Hydrogen Ion Glutaconyl-CoA Water 3-Hydroxy-3- methylglutaryl- CoA Succinate Succinyl-CoA Oxidized flavoprotein Reduced flavoprotein SLC13A3 Citric Acid Cycle Biotin Metabolism
SLC7A2 SLC25A2 SLC13A3 AASS AASS ALDH7A1 AADAT ECHS1 HADH ACAT1 PIPOX GCDH DHTKD1 ACADM AUH SUGCT Ac-CoA Lys Lys Lys Alysine Alysine 3H 3H NADPH AKG Sacchar NADP H2O H2O NAD Glu NADH NAD H2O AAD NADH AKG Oxoadip Glu 3HB-CoA CrtylCA H2O NAD ActaCoA NADH CoA Pipecol O2 S-TH2C H2O2 GlutCoA CO2 CoA H2O H+ GluCCoA H2O HMG-CoA Succi Suc-CoA Pyr-5'P ThiamPP Lipoamd FAD FAD OF RF SLC13A3 PW000005 PW000013 FAD FAD Inner Mitochondria Membrane Outer Mitochondria Membrane Mitochondrial Intermembrane Space Peroxisome Mitochondria Extracellular Space Intracellular Space Liver Extracellular Space Mitochondrial matrix Liver Hepatic Cell Symptoms include encephalopathy, grimacing, dystonia, metabolic acidosis, and hygroma.
Mitochondria SLC7A2 SLC25A2 SLC13A3 AASS AASS ALDH7A1 AADAT ECHS1 HADH ACAT1 PIPOX GCDH DHTKD1 ACADM AUH SUGCT Ac-CoA Lys Lys Lys Alysine Alysine 3H 3H NADPH AKG Sacchar NADP H2O H2O NAD Glu NADH NAD H2O AAD NADH AKG Oxoadip Glu 3HB-CoA CrtylCA H2O NAD ActaCoA NADH CoA Pipecol O2 S-TH2C H2O2 GlutCoA CO2 CoA H2O H+ GluCCoA H2O HMG-CoA Succi Suc-CoA OF RF SLC13A3 PW000005 PW000013