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Cationic amino acid transporter 2 Mitochondrial ornithine transporter 1 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 Pipecolic acid and sarcosine oxidase Short-chain specific acyl-CoA dehydrogenase, mitochondrial Oxoglutarate dehydrogenase complex Acetyl-CoA L-Lysine L-Lysine L-Lysine NADPH Oxoglutaric acid Saccharopine NADP H2O H2O NAD Allysine L-Glutamic acid NADH NAD H2O Accumulation Aminoadipic acid NADH Oxoglutaric acid Accumulation Oxoadipic acid L-Glutamic acid 3-Hydroxybutyryl-CoA Crotonoyl-CoA H2O NAD Acetoacetyl-CoA NADH CoA (S)-2,3,4,5- Tetrahydropiperidine-2- carboxylate H2O L-Pipecolic acid O2 H2O2 Glutaryl-CoA CO2 CoA Pyridoxal 5'-phosphate FAD FAD Thiamine pyrophosphate Lipoamide FAD Citric Acid Cycle Biotin Metabolism Inner Mitochondria Membrane Outer Mitochondria Membrane Mitochondrial Intermembrane Space Peroxisome Mitochondria Extracellular Space Intracellular Space Liver Extracellular Space Mitochondrial matrix
Mitochondria Slc7a2 Slc25a15 Aass Aass Aldh7a1 Aadat Echs1 Hadh Acat1 Pipox Acads Dhtkd1 Acetyl-CoA L-Lysine L-Lysine L-Lysine NADPH Oxoglutaric acid Saccharopine NADP Water Water NAD Allysine L-Glutamic acid NADH NAD Water Accumulation Aminoadipic acid NADH Oxoglutaric acid Accumulation Oxoadipic acid L-Glutamic acid 3- Hydroxybutyryl- CoA Crotonoyl-CoA Water NAD Acetoacetyl-CoA NADH Coenzyme A (S)-2,3,4,5- Tetrahydropiperidine- 2-carboxylate Water L-Pipecolic acid Oxygen Hydrogen peroxide Glutaryl-CoA Carbon dioxide Coenzyme A Citric Acid Cycle Biotin Metabolism