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Mitochondrial ornithine transporter 1 Argininosuccinate synthase Argininosuccinate lyase Carbamoyl- phosphate synthase [ammonia], mitochondrial Glutamate dehydrogenase 1, mitochondrial Ornithine aminotransferase, mitochondrial Delta-1- pyrroline-5- carboxylate dehydrogenase, mitochondrial Delta-1- pyrroline-5- carboxylate dehydrogenase, mitochondrial Proline dehydrogenase 1, mitochondrial Pyrroline-5- carboxylate reductase 2 Prolyl 4-hydroxylase subunit alpha-3 Proline dehydrogenase 1, mitochondrial Pyrroline-5- carboxylate reductase 2 Probable arginine--tRNA ligase, mitochondrial Glycine amidinotransferase, mitochondrial Guanidinoacetate N- methyltransferase Creatine kinase B-type Arginase-1 Ornithine carbamoyltransferase, mitochondrial D-amino-acid oxidase D-amino-acid oxidase Delta-1- pyrroline-5- carboxylate dehydrogenase, mitochondrial Proline dehydrogenase 1, mitochondrial Delta-1- pyrroline-5- carboxylate dehydrogenase, mitochondrial Aspartate aminotransferase, cytoplasmic Nitric oxide synthase, brain Bifunctional glutamate/proline- -tRNA ligase Fumaric acid L-Aspartic acid Ornithine L-Arginine Ornithine Citrulline Citrulline ATP AMP PPi Argininosuccinic acid ATP NH3 CO2 H2O ADP Phosphoric acid Carbamoyl phosphate L-Glutamic acid H2O NAD Oxoglutaric acid NADH Oxoglutaric acid L-Glutamic γ-semialdehyde NAD H2O NADH H+ 1-Pyrroline-5-carboxylic acid NAD H2O NADH Accumulation L-Proline NAD NADH Oxoglutaric acid O2 4- Hydroxyproline Succinic acid CO2 Pyrroline hydroxycarboxylic acid NAD NADH ATP AMP PPi Glycine Ornithine Guanidoacetic acid S-Adenosylmethionine S-Adenosylhomocysteine Creatine ATP ADP Phosphocreatine H2O Urea Pi Accumulation D-Proline H2O O2 1-Pyrroline- 2-carboxylic acid NH3 H2O2 H2O O2 1-Pyrroline- 4-hydroxy-2- carboxylate NH3 H2O2 NAD H2O 4-Hydroxy-L- glutamic acid NADPH L-4- Hydroxyglutamate semialdehyde NAD H2O NADPH Oxalacetic acid 4-Hydroxy-2- oxoglutaric acid L-Aspartic acid NADPH O2 N(omega)-Hydroxyarginine Nitric oxide NADP H2O ATP AMP PPi Pyridoxal 5'-phosphate FAD FAD Manganese FAD FAD FAD Pyridoxal 5'-phosphate Heme FAD Flavin Mononucleotide Tetrahydrobiopterin tRNA(Arg) L-Arginyl- tRNA(Arg) tRNA(Pro) L-Prolyl- tRNA(Pro) Citric Acid Cycle Alanine Metabolism D-Arginine and D-Ornithine Metabolism D-Arginine and D-Ornithine Metabolism Urea Cycle Intracellular Space Extracellular Space Mitochondria Lysosome Mitochondria