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CHEBI:16015 ChEBI HMDB00134 HMDB 2.0 Ornithine carbamoyltransferase, mitochondrial 1.0 439153 PubChem-compound CHEBI:27391 ChEBI 50 ChemSpider HMDB03411 HMDB CHEBI:16027 ChEBI HMDB00148 HMDB OXALACETIC_ACID BioCyc Delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial Guanidoacetic acid 559142 ChemSpider Ornithine aminotransferase, mitochondrial Q5T160 UniProt P29475 UniProt BTO:0000759 BRENDA TISSUE ONTOLOGY 5745 ChemSpider 5742 ChemSpider Ornithine carbamoyltransferase, mitochondrial L-ASPARTATE BioCyc 1143 ChemSpider CHEBI:16474 ChEBI Aspartate aminotransferase, cytoplasmic Carbon dioxide UREA BioCyc HMDB00123 HMDB Phosphocreatine L-CITRULLINE BioCyc HMDB00128 HMDB 1.0 CHEBI:16480 ChEBI CHEBI:16240 ChEBI P04181 UniProt L-4-Hydroxyglutamate semialdehyde 1-Pyrroline-5-carboxylic acid L-arginyl-tRNA(Arg) 1.0 C5H9NO4 L-4-Hydroxyglutamate semialdehyde 147.05316 CH4NO5P Carbamoyl phosphate 140.98271 979-92-0 CAS Arginine: Glycine Amidinotransferase Deficiency (AGAT Deficiency) SUC BioCyc Adenosine monophosphate 7664-38-2 CAS 2.0 6.0 1.0 1.0 C00009 KEGG Compound 1159 ChemSpider C00008 KEGG Compound C00007 KEGG Compound C00006 KEGG Compound C05938 KEGG Compound CHEBI:18009 ChEBI C00001 KEGG Compound Q7Z4N8 UniProt CHEBI:18240 ChEBI C05933 KEGG Compound C00005 KEGG Compound C00004 KEGG Compound C00003 KEGG Compound C00002 KEGG Compound 2485-33-8 CAS 496-92-4 CAS Pyrophosphate Carbamoyl-phosphate synthase [ammonia], mitochondrial HMDB01429 HMDB N-(o)-Hydroxyarginine false L-Arginine + Water → Ornithine + Urea LEFT_TO_RIGHT C00019 KEGG Compound 352-97-6 CAS 1.0 ReactionCatalysis2194 ACTIVATION ReactionCatalysis2193 ACTIVATION C00011 KEGG Compound 1.0 C00014 KEGG Compound C00013 KEGG Compound 10102-43-9 CAS 1-Pyrroline-5-carboxylic acid ReactionCatalysis2196 ACTIVATION ReactionCatalysis2195 ACTIVATION 1.0 ReactionCatalysis2198 ACTIVATION ReactionCatalysis2197 ACTIVATION ReactionCatalysis2199 ACTIVATION http://identifiers.org/smpdb/SMP00362 SMPDB H Hydrogen Ion 1.007825 Phosphate 56-65-5 CAS C00021 KEGG Compound C00020 KEGG Compound 53-84-9 CAS C00027 KEGG Compound C00026 KEGG Compound C00025 KEGG Compound CHEBI:26078 ChEBI 1.0 1.0 L-Glutamic acid Oxygen false L-Glutamic acid + NAD + Water → Ammonia + NADH + Oxoglutaric acid LEFT_TO_RIGHT CHEBI:29171 ChEBI CL:0000000 CELL TYPE ONTOLOGY C5H7NO3 1-Pyrroline-4-hydroxy-2-carboxylate 129.04259 C01127 KEGG Compound C00037 KEGG Compound C00036 KEGG Compound SMILES OC(=O)CCC(O)=O L-prolyl-tRNA(Pro) 1.0 L-arginyl-tRNA(Arg) tRNA(Pro) tRNA(Arg) CHEBI:29177 ChEBI SMILES [O-]P([O-])(=O)OP([O-])([O-])=O 4-Hydroxy-L-proline 128566 ChemSpider C00042 KEGG Compound Water C00049 KEGG Compound false 1-Pyrroline-5-carboxylic acid + NAD + 2 Water ↔ L-Glutamic acid + NADH REVERSIBLE SMILES NC1=NC=NC2=C1N=CN2[C@@H]1O[C@H](COP(O)(=O)OP(O)(O)=O)[C@@H](O)[C@H]1O Reaction2298 false L-Proline → D-Proline LEFT_TO_RIGHT 1.0 false D-Proline + Oxygen + Water → 1-Pyrroline-2-carboxylic acid + Ammonia + Hydrogen peroxide LEFT_TO_RIGHT HMDB00538 HMDB CH4N2O Urea 60.032364 784 PubChem-compound false 2 Argininosuccinic acid → Fumaric acid + L-Arginine LEFT_TO_RIGHT FUM BioCyc HMDB06055 HMDB SMILES OC(=O)CC(=O)C(O)=O false Adenosine triphosphate + Citrulline + L-Aspartic acid → Adenosine monophosphate + Argininosuccinic acid + Pyrophosphate LEFT_TO_RIGHT CHEBI:18012 ChEBI C03564 KEGG Compound SMILES N[C@@H](CCCNC(N)=N)C(O)=O 1.0 C00062 KEGG Compound 62461 ChemSpider SMILES OC(=O)C1CCC=N1 CHEBI:29154 ChEBI Adenosine diphosphate BiologicalState14 Homo sapiens, Cell Membrane C10H16N5O13P3 Adenosine triphosphate 506.99576 BiologicalState13 Homo sapiens, Liver, Mitochondrion NAD Pyrophosphate 1.0 HMDB00517 HMDB P50440 UniProt 962 PubChem-compound 31983 ChemSpider Probable arginine--tRNA ligase, mitochondrial C00077 KEGG Compound 2.0 C01165 KEGG Compound CO2 Carbon dioxide 43.98983 Proline dehydrogenase 1, mitochondrial 145742 PubChem-compound C00080 KEGG Compound 2139-03-9 CAS SMILES O=O BiologicalState9 Homo sapiens, Cell, Lysosome BiologicalState8 Homo sapiens, Cell, Cytoplasm BiologicalState3 Homo sapiens, Cell, Mitochondrion BiologicalState4 Homo sapiens, Cell, Mitochondrial Matrix BiologicalState2 Homo sapiens, Cell, Cytosol 14265-44-2 CAS 1.0 9054-77-7 CAS C00086 KEGG Compound 1.0 CPD-1128 BioCyc 644102 PubChem-compound L-Arginine 1.0 C5H7NO5 4-Hydroxy-L-glutamic acid 161.03242 H2O2 Hydrogen peroxide 34.005478 Ammonia NADH BioCyc 1.0 CREATINE BioCyc 29908-03-0 CAS 977 PubChem-compound NADPH Bifunctional glutamate/proline--tRNA ligase 1.0 970 PubChem-compound C5H9NO3 4-Hydroxy-L-proline 131.05824 SMILES OC1CC(N=C1)C(O)=O NAD 3.0 P07814 UniProt 30572 ChemSpider CPD-656 BioCyc 17215925 ChemSpider SMILES N[C@@H](CC(O)C([O-])=O)C([O-])=O Argininosuccinate synthase Carbamoyl-phosphate synthase [ammonia], mitochondrial 4-Hydroxy-L-glutamic acid 21883788 PubChem-compound SMILES OO C10H18N4O6 Argininosuccinic acid 290.12262 H3N Ammonia 17.026548 C10H14N5O7P Adenosine monophosphate 347.06308 599 PubChem-compound P00480 UniProt 1.0 Ammonia SMILES OC1CN[C@@H](C1)C(O)=O Water 51 PubChem-compound C4H9N3O2 Creatine 131.06947 5460078 PubChem-compound CHEBI:15729 ChEBI 167744 ChemSpider SMILES OC1CN=C(C1)C(O)=O 1.0 D-Arginine and D-Ornithine Metabolism SubPathway 274 ChemSpider 272 ChemSpider Ornithine SubPathwayInteraction840 SubPathwayReaction SubPathway840Reaction SubPathwayInteraction841 SubPathwayReaction SubPathway841Reaction Q14353 UniProt SubPathwayInteraction842 SubPathway842Reaction SubPathwayReaction SubPathwayInteraction843 SubPathway843Reaction SubPathwayReaction O4P Phosphate 94.95342 2.0 Adenosine triphosphate 1.0 Q9Y619 UniProt SMILES NC(N)=O Q96C36 UniProt SubPathwayInteraction7 SubPathway7Reaction SubPathwayReaction NADPH BioCyc Citrulline S-Adenosylhomocysteine CPD-6641 BioCyc DELTA1-PYRROLINE_2-CARBOXYLATE BioCyc HMDB06556 HMDB 217 ChemSpider Pyrroline-5-carboxylate reductase 2 58-68-4 CAS 2.0 C4H10N3O5P Phosphocreatine 211.03581 SMILES [O-]P([O-])([O-])=O C04281 KEGG Compound C04282 KEGG Compound CHEBI:30915 ChEBI 4-Hydroxy-L-proline l-delta(1)-pyrroline_5-carboxylate BioCyc C2H5NO2 Glycine 75.03203 Ornithine SubPathwayOutput GO:0005764 GENE ONTOLOGY 344-25-2 CAS Arginase-1 Liver C3H7N3O2 Guanidoacetic acid 117.053825 GO:0005759 GENE ONTOLOGY C4H4O4 Fumaric acid 116.010956 586 PubChem-compound CHEBI:15741 ChEBI 587 PubChem-compound SMILES O=C=O D-4-HYDROXY-2-KETO-GLUTARATE BioCyc 1110 PubChem-compound C21H29N7O14P2 NADH 665.12476 Glycine amidinotransferase, mitochondrial 1.0 5957 PubChem-compound 2.0 HMDB01096 HMDB 1.0 1.0 GO:0005739 GENE ONTOLOGY GO:0005737 GENE ONTOLOGY 2.0 1.0 16059 ChemSpider 147-85-3 CAS C5H6O6 4-Hydroxy-2-oxoglutaric acid 162.01643 Creatine 0.0 P05089 UniProt 2.0 1.0 NADH 1.0 1.0 5682 ChemSpider false Adenosine triphosphate + Creatine → Adenosine diphosphate + Phosphocreatine LEFT_TO_RIGHT Mitochondrial Matrix 14000-31-8 CAS Urea L-Glutamic gamma-semialdehyde Prolyl 4-hydroxylase subunit alpha-3 P30038 UniProt 67-07-2 CAS 5675 ChemSpider 1078 ChemSpider 389057 ChemSpider NITRIC-OXIDE BioCyc Carbon dioxide CHEBI:1372 ChEBI SMILES NC(=O)OP(O)(O)=O Cell HMDB00191 HMDB 22573-88-2 CAS Creatine kinase B-type 28782 PubChem-compound false L-Glutamic gamma-semialdehyde + NAD + Water → Hydrogen Ion + L-Glutamic acid + NADH LEFT_TO_RIGHT 1.0 C5H9NO4 L-Glutamic acid 147.05316 Creatine kinase B-type 33032 PubChem-compound L-Arginine SubPathwayInput Transport39 false Ornithine (→), Citrulline (←) Transport: Homo sapiens, Cell, Cytoplasm to Homo sapiens, Cell, Mitochondrial Matrix LEFT_TO_RIGHT 4.0 HMDB02142 HMDB 7782-44-7 CAS CHEBI:30923 ChEBI Protein-hydroxyprolines BioCyc 9606 TAXONOMY Nitric oxide 2-KETOGLUTARATE BioCyc Guanidinoacetate N-methyltransferase 1.0 1176 PubChem-compound HMDB02111 HMDB 110-15-6 CAS Phosphoric acid Nitric oxide synthase, brain Oxoglutaric acid false 2 L-Arginine + 3 NADPH + 4 Oxygen → 2 N-(o)-Hydroxyarginine + 3 NADP + 2 Nitric oxide + 4 Water LEFT_TO_RIGHT PW000019 PathWhiz Reaction2305 false 4-Hydroxy-L-glutamic acid + Oxalacetic acid → 4-Hydroxy-2-oxoglutaric acid + L-Aspartic acid LEFT_TO_RIGHT false 4-Hydroxy-L-glutamic acid ↔ L-4-Hydroxyglutamate semialdehyde REVERSIBLE C6H14N4O3 N-(o)-Hydroxyarginine 190.1066 false 4-Hydroxy-L-glutamic acid + NAD + Water → L-4-Hydroxyglutamate semialdehyde + NADPH LEFT_TO_RIGHT false 4-Hydroxy-L-proline + Oxygen + Water → 1-Pyrroline-4-hydroxy-2-carboxylate + Ammonia + Hydrogen peroxide LEFT_TO_RIGHT false NAD + Pyrroline hydroxycarboxylic acid + Water → 4-Hydroxy-L-glutamic acid + NADPH LEFT_TO_RIGHT CHEBI:15377 ChEBI Cell Membrane 1.0 CHEBI:15379 ChEBI CHEBI:16467 ChEBI CHEBI:15378 ChEBI SMILES [H+] SMILES NC1=NC=NC2=C1N=CN2[C@@H]1O[C@H](COP(O)(=O)OP(O)(=O)OP(O)(O)=O)[C@@H](O)[C@H]1O 51-35-4 CAS P31327 UniProt 389257 ChemSpider NADPH C00763 KEGG Compound Ornithine aminotransferase, mitochondrial CHEBI:30744 ChEBI Carbamoyl phosphate HMDB01487 HMDB PW000001 PathWhiz SMILES N[C@@H](C[C@@H](O)C=O)C(O)=O PW000005 PathWhiz 1196 PubChem-compound C00533 KEGG Compound 1.0 2.0 1.0 1.0 HMDB02104 HMDB 74-79-3 CAS CHEBI:16680 ChEBI HMDB00162 HMDB C00300 KEGG Compound 2906-39-0 CAS L-Proline 69248 PubChem-compound 1.0 3.0 Prolyl 4-hydroxylase subunit alpha-3 HMDB00254 HMDB 1.0 6022 PubChem-compound 7722-84-1 CAS 1.0 Citrulline 6262 PubChem-compound 937 ChemSpider 127983 ChemSpider HMDB00250 HMDB CHEBI:16134 ChEBI HMDB01341 HMDB L-Aspartic acid SubPathwayOutput 110-17-8 CAS Arginase-1 C00327 KEGG Compound 1.0 tRNA(Arg) 389693 ChemSpider Pyrroline hydroxycarboxylic acid 389691 ChemSpider ATP BioCyc CHEBI:17232 ChEBI Aspartate aminotransferase, cytoplasmic D-Proline Hydrogen peroxide Aspartate aminotransferase, cytoplasmic http://identifiers.org/smpdb/SMP00036 SMPDB PRO BioCyc Glycine amidinotransferase, mitochondrial CHEBI:17203 ChEBI C15H23N6O5S S-Adenosylmethionine 399.14505 1-Pyrroline-2-carboxylic acid 1.0 CHEBI:16352 ChEBI 1.0 Pyrroline hydroxycarboxylic acid 952 ChemSpider 440046 PubChem-compound HMDB59597 HMDB Proline dehydrogenase 1, mitochondrial 440282 PubChem-compound 5858 ChemSpider ARG BioCyc Adenosine monophosphate C00581 KEGG Compound 945 ChemSpider 16950 PubChem-compound 1010 ChemSpider 1.0 Pyrroline-5-carboxylate reductase 2 1.0 PPI BioCyc http://identifiers.org/smpdb/SMP00057 SMPDB Arginase-1 http://identifiers.org/smpdb/SMP00055 SMPDB HMDB01301 HMDB HMDB00214 HMDB HMDB00217 HMDB SMILES C[S+](CC[C@H](N)C(O)=O)C[C@H]1O[C@H]([C@H](O)[C@@H]1O)N1C=NC2=C(N)N=CN=C12 Cytosol C14H20N6O5S S-Adenosylhomocysteine 384.12158 193305 PubChem-compound 1.0 HMDB00208 HMDB false Glycine + L-Arginine ↔ Guanidoacetic acid + Ornithine REVERSIBLE false Guanidoacetic acid + S-Adenosylmethionine → Creatine + S-Adenosylhomocysteine LEFT_TO_RIGHT HMDB00221 HMDB HMDB00223 HMDB C02305 KEGG Compound C00122 KEGG Compound 1.0 1030 ChemSpider CHEBI:18361 ChEBI NADH CHEBI:18366 ChEBI CHEBI:18367 ChEBI 1032 ChemSpider 1.0 Fumaric acid SubPathwayInput Carbamoyl phosphate C6H13N3O3 Citrulline 175.09569 372-75-8 CAS C03406 KEGG Compound C21H28N7O14P2 NAD 664.11694 Bifunctional glutamate/proline--tRNA ligase L-Arginine 44367445 PubChem-compound Adenosine triphosphate 2.0 Prolyl 4-hydroxylase L-Proline PW000084 PathWhiz NAD(P) BioCyc SMILES NC(CCC\N=C(/N)NO)C(O)=O 388301 ChemSpider Nitric-oxide synthase, brain C00148 KEGG Compound C5H9NO2 D-Proline 115.06333 9367 ChemSpider 328-50-7 CAS SMILES NC(=O)C1=CN(C=CC1)[C@@H]1O[C@H](CO[P@](O)(=O)O[P@](O)(=O)OC[C@H]2O[C@H]([C@H](O)[C@@H]2O)N2C=NC3=C(N)N=CN=C23)[C@@H](O)[C@H]1O Argininosuccinate lyase AMP BioCyc CHEBI:7101 ChEBI GUANIDOACETIC_ACID BioCyc Oxalacetic acid HYDROGEN-PEROXIDE BioCyc Adenosine diphosphate HMDB01511 HMDB Argininosuccinate synthase 1.0 4-Hydroxy-L-glutamic acid C00169 KEGG Compound C01015 KEGG Compound Ornithine aminotransferase, mitochondrial 763 ChemSpider 1.0 Argininosuccinate lyase C5H7NO2 1-Pyrroline-2-carboxylic acid 113.047676 1.0 1.0 1.0 SMILES OC(CC(=O)C(O)=O)C(O)=O 10197150 ChemSpider Pyrroline hydroxycarboxylic acid 1.0 Ammonia 7732-18-5 CAS CHEBI:16199 ChEBI Glycine amidinotransferase, mitochondrial CHEBI:17287 ChEBI 61-19-8 CAS NAD SMILES NC1=C2N=CN([C@@H]3O[C@H](COP(O)(O)=O)[C@@H](O)[C@H]3O)C2=NC=N1 Creatine kinase B-type D-amino-acid oxidase SMILES N[C@@H](CCCNC(=N)N[C@@H](CC(O)=O)C(O)=O)C(O)=O AMMONIA BioCyc SMILES N 56-86-0 CAS 590-55-6 CAS Glutamate dehydrogenase 1, mitochondrial 2.0 CHEBI:17053 ChEBI 56-40-6 CAS NO Nitric oxide 29.997988 2387-71-5 CAS SMILES CN(CC(O)=O)C(N)=N HMDB01967 HMDB 2.0 C5H9NO2 L-Proline 115.06333 5800 ChemSpider 4.0 979 ChemSpider Argininosuccinate synthase 730 ChemSpider Water H3O4P Phosphoric acid 97.9769 4-Hydroxy-2-oxoglutaric acid SMILES N[C@@H](CCC(O)=O)C(O)=O 58-64-0 CAS 1.0 1.0 Mitochondrial ornithine transporter 1 C04322 KEGG Compound D-amino-acid oxidase Pyrroline-5-carboxylate reductase 2 Nitric oxide synthase, brain Ornithine carbamoyltransferase, mitochondrial C4H7NO4 L-Aspartic acid 133.0375 1.0 L-GLUTAMATE_GAMMA-SEMIALDEHYDE BioCyc 57-00-1 CAS 1.0 6083 PubChem-compound Argininosuccinate lyase C5H9NO3 L-Glutamic gamma-semialdehyde 131.05824 25246222 PubChem-compound 22833512 PubChem-compound L-Aspartic acid SMILES CN(CC(O)=O)C(=N)NP(O)(O)=O Argininosuccinic acid H2O Water 18.010565 Oxygen SMILES NCC(O)=O P12277 UniProt Delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial NAD BioCyc 0.0 Guanidinoacetate N-methyltransferase 56-84-8 CAS Glutamate dehydrogenase 1, mitochondrial Glutamate dehydrogenase 1, mitochondrial 1.0 SMILES OC(=O)\C=C\C(O)=O 743 ChemSpider 3.0 P04424 UniProt SMILES NC(=N)NCC(O)=O D-amino-acid oxidase ReactionCatalysis153 ACTIVATION ReactionCatalysis18 ACTIVATION ReactionCatalysis151 ACTIVATION C4H4O5 Oxalacetic acid 132.00587 ReactionCatalysis152 ACTIVATION SMILES N[C@@H](CCC=O)C(O)=O ReactionCatalysis150 ACTIVATION Adenosine triphosphate ReactionCatalysis12 ACTIVATION 1.0 C5H7NO2 1-Pyrroline-5-carboxylic acid 113.047676 SMILES O 57-13-6 CAS C6H14N4O2 L-Arginine 174.11168 4.0 ReactionCatalysis146 ACTIVATION ReactionCatalysis147 ACTIVATION Reaction649 true L-Glutamic gamma-semialdehyde ↔ 1-Pyrroline-5-carboxylic acid REVERSIBLE ReactionCatalysis145 ACTIVATION 6992098 PubChem-compound ReactionCatalysis143 ACTIVATION ReactionCatalysis140 ACTIVATION ReactionCatalysis141 ACTIVATION 1004 PubChem-compound 1.0 328-42-7 CAS CHEBI:15846 ChEBI P00367 UniProt ADP BioCyc ReactionCatalysis139 ACTIVATION ReactionCatalysis137 ACTIVATION HMDB00939 HMDB ReactionCatalysis138 ACTIVATION ReactionCatalysis38 ACTIVATION 4.0 1.0 1.0 Lysosome 53-59-8 CAS Reaction658 true Pyrroline hydroxycarboxylic acid ↔ L-4-Hydroxyglutamate semialdehyde REVERSIBLE false Ornithine + Oxoglutaric acid ↔ L-Glutamic acid + L-Glutamic gamma-semialdehyde REVERSIBLE false 4-Hydroxy-L-proline ↔ Pyrroline hydroxycarboxylic acid REVERSIBLE 440849 PubChem-compound SMILES OC(=O)[C@H]1CCCN1 1038 PubChem-compound CHEBI:27809 ChEBI CHEBI:36761 ChEBI 4.0 GO:0005829 GENE ONTOLOGY C5H6O5 Oxoglutaric acid 146.02153 false 4-Hydroxy-L-proline + NAD → NADH + Pyrroline hydroxycarboxylic acid LEFT_TO_RIGHT HMDB00902 HMDB Reaction653 false Adenosine triphosphate + L-Proline + tRNA(Pro) → Adenosine monophosphate + L-prolyl-tRNA(Pro) + Pyrophosphate LEFT_TO_RIGHT HMDB00904 HMDB false 1-Pyrroline-5-carboxylic acid ↔ L-Proline REVERSIBLE false L-Proline + NAD → 1-Pyrroline-5-carboxylic acid + NADH LEFT_TO_RIGHT CHEBI:16908 ChEBI L-ERYTHRO-4-HYDROXY-GLUTAMATE BioCyc 2.0 Delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial Guanidoacetic acid 70-26-8 CAS ADENOSYL-HOMO-CYS BioCyc ReactionCatalysis41 ACTIVATION 6026 ChemSpider 1.0 SMILES [O-]C(=O)C1=[NH+]CCC1 false Carbamoyl phosphate + Ornithine → Citrulline + Phosphate LEFT_TO_RIGHT C21H30N7O17P3 NADPH 745.0911 Mitochondrion C21H29N7O17P3 NADP 744.08325 C5H12N2O2 Ornithine 132.08987 false Adenosine triphosphate + L-Arginine + tRNA(Arg) → Adenosine monophosphate + L-arginyl-tRNA(Arg) + Pyrophosphate LEFT_TO_RIGHT CHEBI:16919 ChEBI O43272 UniProt GLY BioCyc 1187-99-1 CAS 579 ChemSpider Water SMILES OC(=O)CCC(=O)C(O)=O HMDB02070 HMDB GO:0005886 GENE ONTOLOGY 124-38-9 CAS CHEBI:15414 ChEBI NADP C03079 KEGG Compound 1.0 567 ChemSpider 440851 PubChem-compound SMILES NC(=O)C1=C[N+](=CC=C1)[C@@H]1O[C@H](CO[P@](O)(=O)O[P@](O)(=O)OC[C@H]2O[C@H]([C@H](OP(O)(O)=O)[C@@H]2O)N2C=NC3=C(N)N=CN=C23)[C@@H](O)[C@H]1O 566 ChemSpider 1.0 SMILES NCCC[C@H](N)C(O)=O 5257127 PubChem-compound C5H7NO3 Pyrroline hydroxycarboxylic acid 129.04259 SMILES NC(=O)C1=CN(C=CC1)[C@H]1O[C@@H](COP(O)(=O)OP(O)(=O)OC[C@@H]2O[C@@H]([C@@H](OP(O)(O)=O)[C@H]2O)N2C=NC3=C(N)N=CN=C23)[C@H](O)[C@@H]1O 7664-41-7 CAS GLT BioCyc Oxygen 53-57-6 CAS SMILES OC(=O)[C@@H]1CCCN1 S-ADENOSYLMETHIONINE BioCyc 222 PubChem-compound Hydrogen Ion SMILES OP(O)(O)=O 1.0 O7P2 Pyrophosphate 173.91193 6082 ChemSpider C4H6O4 Succinic acid 118.02661 Ornithine C10H15N5O10P2 Adenosine diphosphate 427.02942 SMILES N[C@@H](CC(O)=O)C(O)=O HMDB03125 HMDB Delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial 1.0 SMILES N[C@@H](CCCNC(N)=O)C(O)=O 10.0 TransportCatalysis12 ACTIVATION 3.0 CPD-8587 BioCyc SMILES NC(=O)C1=C[N+](=CC=C1)[C@@H]1O[C@H](COP(O)(=O)OP(O)(=O)OC[C@H]2O[C@H]([C@H](O)[C@@H]2O)N2C=NC3=C2N=CN=C3N)[C@@H](O)[C@H]1O HMDB02273 HMDB HMDB06875 HMDB HMDB01185 HMDB CHEBI:15682 ChEBI 4-Hydroxy-L-proline Proline dehydrogenase 1, mitochondrial 1.0 Mitochondrial ornithine transporter 1 1-Pyrroline-4-hydroxy-2-carboxylate HMDB03378 HMDB CARBAMOYL-P BioCyc 2.0 P14920 UniProt HMDB04224 HMDB Probable arginine--tRNA ligase, mitochondrial Cytoplasm 1.0 1.0 CHEBI:15422 ChEBI Glycine 1.0 8988 PubChem-compound 388299 ChemSpider O2 Oxygen 31.98983 1.0 PYRROLINE-HYDROXY-CARBOXYLATE BioCyc Delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial CREATINE-P BioCyc Water CHEBI:16526 ChEBI CHEBI:16761 ChEBI CHEBI:15428 ChEBI L-Proline HMDB02234 HMDB CHEBI:16338 ChEBI 145068 PubChem-compound 1059 PubChem-compound HMDB00051 HMDB HMDB00052 HMDB HMDB00294 HMDB Oxoglutaric acid 5893 PubChem-compound false L-Proline + Oxoglutaric acid + Oxygen → 4-Hydroxy-L-proline + Carbon dioxide + Succinic acid LEFT_TO_RIGHT 1.0 1.0 CHEBI:16344 ChEBI 278 PubChem-compound CHEBI:16349 ChEBI L-prolyl-tRNA(Pro) false 2 Adenosine triphosphate + Ammonia + Carbon dioxide + Water → 2 Adenosine diphosphate + Carbamoyl phosphate + Phosphoric acid LEFT_TO_RIGHT SMILES [N]=O 5886 PubChem-compound HMDB00064 HMDB 1.0 CHEBI:17672 ChEBI 280 PubChem-compound 2.0 1.0 P00966 UniProt ReactionCatalysis136 ACTIVATION ReactionCatalysis133 ACTIVATION CHEBI:16313 ChEBI D-Proline HMDB01369 HMDB SMILES N[C@@H](CCSC[C@H]1O[C@H]([C@H](O)[C@@H]1O)N1C=NC2=C(N)N=CN=C12)C(O)=O 4573749 ChemSpider Succinic acid 1.0 Alanine Metabolism SubPathway 3946848 PubChem-compound Citric Acid Cycle SubPathway HMDB00045 HMDB HMDB01377 HMDB 1061 PubChem-compound 8640 ChemSpider 24762165 PubChem-compound 1.0 tRNA(Pro) L-ORNITHINE BioCyc S-Adenosylmethionine P17174 UniProt 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 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 PP i Argininosuccinic acid ATP