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Low affinity cationic amino acid transporter 2 Mitochondrial ornithine transporter 2 Unknown 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 Acetyl-CoA L-Lysine L-Lysine L-Lysine Allysine Allysine 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 Pyridoxal 5'-phosphate Thiamine pyrophosphate Lipoamide FAD 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 developmental delay, spastic diplegia, seizures, rigidity, coma, episodic vomiting, and diarrhea. Most experience no symptoms
Mitochondria SLC7A2 SLC25A2 Unknown AASS AASS ALDH7A1 AADAT ECHS1 HADH ACAT1 PIPOX GCDH DHTKD1 Acetyl-CoA L-Lysine L-Lysine L-Lysine Allysine Allysine 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 Citric Acid Cycle Biotin Metabolism
SLC7A2 SLC25A2 AASS AASS ALDH7A1 AADAT ECHS1 HADH ACAT1 PIPOX GCDH DHTKD1 Ac-CoA Lys Lys Lys Alysine Alysine 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 Pyr-5'P ThiamPP Lipoamd FAD 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 developmental delay, spastic diplegia, seizures, rigidity, coma, episodic vomiting, and diarrhea. Most experience no symptoms
Mitochondria SLC7A2 SLC25A2 AASS AASS ALDH7A1 AADAT ECHS1 HADH ACAT1 PIPOX GCDH DHTKD1 Ac-CoA Lys Lys Lys Alysine Alysine 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 PW000005 PW000013