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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 PP i Argininosuccinic acid ATP NH 3 CO 2 H 2 O ADP Phosphoric acid Carbamoyl phosphate L-Glutamic acid H 2 O NAD Oxoglutaric acid NADH Oxoglutaric acid L-Glutamic γ-semialdehyde NAD H 2 O NADH H + 1-Pyrroline-5-carboxylic acid NAD H 2 O NADH Accumulation L-Proline NAD NADH Oxoglutaric acid O 2 Accumulation 4- Hydroxyproline Succinic acid CO 2 Pyrroline hydroxycarboxylic acid NAD NADH ATP AMP PP i Glycine Ornithine Guanidoacetic acid S-Adenosylmethionine S-Adenosylhomocysteine Creatine ATP ADP Phosphocreatine H 2 O Urea P i Accumulation D-Proline H 2 O O 2 1-Pyrroline- 2-carboxylic acid NH 3 H 2 O 2 H 2 O O 2 1-Pyrroline- 4-hydroxy-2- carboxylate NH 3 H 2 O 2 NAD H 2 O 4-Hydroxy-L- glutamic acid NADPH L-4- Hydroxyglutamate semialdehyde NAD H 2 O NADPH Oxalacetic acid 4-Hydroxy-2- oxoglutaric acid L-Aspartic acid NADPH O 2 N(omega)-Hydroxyarginine Nitric oxide NADP H 2 O ATP AMP PP i 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