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Mitochondrial uncoupling protein 3 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 Bifunctional glutamate/proline- -tRNA ligase 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 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 ATP AMP PPi Oxoglutaric acid O2 Accumulation 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 Pyridoxal 5'-phosphate FAD FAD Manganese FAD FAD FAD Pyridoxal 5'-phosphate Heme FAD Flavin Mononucleotide Tetrahydrobiopterin tRNA(Pro) L-Prolyl- tRNA(Pro) tRNA(Arg) L-Arginyl- tRNA(Arg) 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
Mitochondria Ucp3 Ass1 Asl Cps1 Glud1 Oat Aldh4a1 Aldh4a1 Prodh Pycr2 Eprs P4ha3 Prodh Pycr2 Rars2 Gatm Gamt Ckb Arg1 Otc Dao Dao Aldh4a1 Prodh Aldh4a1 Got1 Nos1 Fumaric acid L-Aspartic acid Ornithine L-Arginine Ornithine Citrulline Citrulline 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 Accumulation L-Proline NAD NADH Adenosine triphosphate Adenosine monophosphate Pyrophosphate Oxoglutaric acid Oxygen Accumulation 4- Hydroxyproline Succinic acid Carbon dioxide Pyrroline hydroxycarboxylic acid NAD NADH Adenosine triphosphate Adenosine monophosphate Pyrophosphate Glycine Ornithine Guanidoacetic acid S- Adenosylmethionine S- Adenosylhomocysteine Creatine Adenosine triphosphate Adenosine diphosphate Phosphocreatine Water Urea Phosphate Accumulation 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 tRNA(Pro) L-Prolyl- tRNA(Pro) tRNA(Arg) L-Arginyl- tRNA(Arg) Citric Acid Cycle Alanine Metabolism D-Arginine and D-Ornithine Metabolism D-Arginine and D-Ornithine Metabolism
Ucp3 Ass1 Asl Cps1 Glud1 Oat Aldh4a1 Aldh4a1 Prodh Pycr2 Eprs P4ha3 Prodh Pycr2 Rars2 Gatm Gamt Ckb Arg1 Otc Dao Dao Aldh4a1 Prodh Aldh4a1 Got1 Nos1 Fumarat Asp Ornithi Arg Ornithi Citruln Citruln 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 Accumulation Pro NAD NADH ATP AMP PPi AKG O2 Accumulation 4-HPro Succini CO2 PyroHcA NAD NADH ATP AMP PPi Gly Ornithi GIAA SAMe SAH Creatin ATP ADP Phosfcr H2O Urea Pi Accumulation 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 Pyr-5'P FAD FAD Mn2+ FAD FAD FAD Pyr-5'P Heme FAD FlvnMnt BH4 TRNAPRO LPTP TRNAARG LATA PW000005 PW000001 PW000019 PW000019 Urea Cycle Intracellular Space Extracellular Space Mitochondria Lysosome Mitochondria
Mitochondria Ucp3 Ass1 Asl Cps1 Glud1 Oat Aldh4a1 Aldh4a1 Prodh Pycr2 Eprs P4ha3 Prodh Pycr2 Rars2 Gatm Gamt Ckb Arg1 Otc Dao Dao Aldh4a1 Prodh Aldh4a1 Got1 Nos1 Fumarat Asp Ornithi Arg Ornithi Citruln Citruln 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 Accumulation Pro NAD NADH ATP AMP Ppi AKG O2 Accumulation 4-HPro Succini CO2 PyroHcA NAD NADH ATP AMP Ppi Gly Ornithi GIAA SAMe SAH Creatin ATP ADP Phosfcr H2O Urea Pi Accumulation 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 TRNAPRO LPTP TRNAARG LATA PW000005 PW000001 PW000019 PW000019