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Mitochondrial ornithine transporter 1 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 Accumulation Ornithine L-Arginine Citrulline Citrulline Accumulation Ornithine 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 L-Proline NAD NADH Oxoglutaric acid O2 4-Hydroxyproline Succinic acid CO2 Pyrroline hydroxycarboxylic acid NAD NADH ATP AMP PPi Glycine Accumulation Ornithine Guanidoacetic acid S-Adenosylmethionine S-Adenosylhomocysteine Creatine ATP ADP Phosphocreatine H2O Urea Pi 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 NO NADP H2O ATP AMP PPi FAD Manganese Pyridoxal 5'-phosphate 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 Pyridoxal 5'-phosphate FAD Heme Flavin Mononucleotide Tetrahydrobiopterin FAD FAD FAD FAD Urea Cycle Cytosol Extracellular Space Mitochondria Lysosome Mitochondria Mitochondrial Matrix
Mitochondria SLC25A15 SLC25A15 ASS1 ASL CPS1 GLUD1 OAT ALDH4A1 ALDH4A1 PRODH PYCR2 P4HA3 PRODH PYCR2 RARS2 GATM GAMT CKB ARG1 OTC DAO DAO ALDH4A1 PRODH ALDH4A1 GOT1 NOS1 EPRS Fumaric acid L-Aspartic acid Accumulation Ornithine L-Arginine Citrulline Citrulline Accumulation Ornithine Adenosine triphosphate Adenosine monophosphate Pyrophosphate Argininosuccinic acid Adenosine triphosphate Ammonia Carbon dioxide Water Adenosine diphosphate Phosphoric acid Carbamoyl phosphate L-Glutamic acid Water NAD Oxoglutaric acid NADH Oxoglutaric acid L-Glutamic γ-semialdehyde NAD Water NADH Hydrogen Ion 1-Pyrroline- 5-carboxylic acid NAD Water NADH L-Proline NAD NADH Oxoglutaric acid Oxygen 4- Hydroxyproline Succinic acid Carbon dioxide Pyrroline hydroxycarboxylic acid NAD NADH Adenosine triphosphate Adenosine monophosphate Pyrophosphate Glycine Accumulation Ornithine Guanidoacetic acid S- Adenosylmethionine S- Adenosylhomocysteine Creatine Adenosine triphosphate Adenosine diphosphate Phosphocreatine Water Urea Phosphate D-Proline Water Oxygen 1-Pyrroline- 2-carboxylic acid Ammonia Hydrogen peroxide Water Oxygen 1-Pyrroline- 4-hydroxy-2- carboxylate Ammonia Hydrogen peroxide NAD Water 4-Hydroxy-L- glutamic acid NADPH L-4- Hydroxyglutamate semialdehyde NAD Water NADPH Oxalacetic acid 4-Hydroxy-2- oxoglutaric acid L-Aspartic acid NADPH Oxygen N(omega)- Hydroxyarginine Nitric oxide NADP Water Adenosine triphosphate Adenosine monophosphate Pyrophosphate 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
SLC25A15 SLC25A15 ASS1 ASL CPS1 GLUD1 OAT ALDH4A1 ALDH4A1 PRODH PYCR2 P4HA3 PRODH PYCR2 RARS2 GATM GAMT CKB ARG1 OTC DAO DAO ALDH4A1 PRODH ALDH4A1 GOT1 NOS1 EPRS Fumarat Asp Accumulation Ornithi Arg Citruln Citruln Accumulation Ornithi ATP AMP PPi ASA ATP NH3 CO2 H2O ADP PhosfrA CarbmP Glu H2O NAD AKG NADH AKG L-Gl-GA NAD H2O NADH H+ 1P5CA NAD H2O NADH Pro NAD NADH AKG O2 4-HPro Succini CO2 PyroHcA NAD NADH ATP AMP PPi Gly Accumulation Ornithi GIAA SAMe SAH Creatin ATP ADP Phosfcr H2O Urea Pi D-Pro H2O O2 1Pyr2C NH3 H2O2 H2O O2 1P4H2C NH3 H2O2 NAD H2O 4HLG NADPH 44H5OLN NAD H2O NADPH Oaa 2O4H Asp NADPH O2 NωH NO NADP H2O ATP AMP PPi FAD Mn2+ Pyr-5'P TRNAARG LATA TRNAPRO LPTP PW000005 PW000001 PW000019 PW000019 Pyr-5'P FAD Heme FlvnMnt BH4 FAD FAD FAD FAD Urea Cycle Cytosol Extracellular Space Mitochondria Lysosome Mitochondria Mitochondrial Matrix
Mitochondria SLC25A15 SLC25A15 ASS1 ASL CPS1 GLUD1 OAT ALDH4A1 ALDH4A1 PRODH PYCR2 P4HA3 PRODH PYCR2 RARS2 GATM GAMT CKB ARG1 OTC DAO DAO ALDH4A1 PRODH ALDH4A1 GOT1 NOS1 EPRS Fumarat Asp Accumulation Ornithi Arg Citruln Citruln Accumulation Ornithi ATP AMP Ppi ASA ATP NH3 CO2 H2O ADP PhosfrA CarbmP Glu H2O NAD AKG NADH AKG L-Gl-GA NAD H2O NADH H+ 1P5CA NAD H2O NADH Pro NAD NADH AKG O2 4-HPro Succini CO2 PyroHcA NAD NADH ATP AMP Ppi Gly Accumulation Ornithi GIAA SAMe SAH Creatin ATP ADP Phosfcr H2O Urea Pi D-Pro H2O O2 1Pyr2C NH3 H2O2 H2O O2 1P4H2C NH3 H2O2 NAD H2O 4HLG NADPH 44H5OLN NAD H2O NADPH Oaa 2O4H Asp NADPH O2 NωH NO NADP H2O ATP AMP Ppi TRNAARG LATA TRNAPRO LPTP PW000005 PW000001 PW000019 PW000019