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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 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 3.5.3.1 false 3.5.3.1 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 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 1.4.1.3 false 1.4.1.3 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 1.2.1.88 false 1.2.1.88 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 1.4.3.3 false 1.4.3.3 D-Proline + Oxygen + Water → 1-Pyrroline-2-carboxylic acid + Ammonia + Hydrogen peroxide LEFT_TO_RIGHT HMDB00538 HMDB CH4N2O Urea 60.032364 784 PubChem-compound 4.3.2.1 false 4.3.2.1 2 Argininosuccinic acid → Fumaric acid + L-Arginine LEFT_TO_RIGHT FUM BioCyc HMDB06055 HMDB SMILES OC(=O)CC(=O)C(O)=O 6.3.4.5 false 6.3.4.5 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 C10H16N5O13P3 Adenosine triphosphate 506.99576 BiologicalState13 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 BiologicalState8 BiologicalState3 BiologicalState4 BiologicalState2 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 1.0 274 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 2.7.3.2 false 2.7.3.2 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 1.2.1.88 false 1.2.1.88 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 1.14.13.39 false 1.14.13.39 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 1.5.5.2 false 1.5.5.2 4-Hydroxy-L-glutamic acid ↔ L-4-Hydroxyglutamate semialdehyde REVERSIBLE C6H14N4O3 N-(o)-Hydroxyarginine 190.1066 1.2.1.88 false 1.2.1.88 4-Hydroxy-L-glutamic acid + NAD + Water → L-4-Hydroxyglutamate semialdehyde + NADPH LEFT_TO_RIGHT 1.4.3.3 false 1.4.3.3 4-Hydroxy-L-proline + Oxygen + Water → 1-Pyrroline-4-hydroxy-2-carboxylate + Ammonia + Hydrogen peroxide LEFT_TO_RIGHT 1.2.1.88 false 1.2.1.88 NAD + Pyrroline hydroxycarboxylic acid + Water → 4-Hydroxy-L-glutamic acid + NADPH LEFT_TO_RIGHT CHEBI:15377 ChEBI Cell Membrane 1.0 CHEBI:15379 ChEBI PW000010 PathWhiz 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 Arginine and Proline Metabolism 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 SMP00020 SMPDB 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 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 SMP00057 SMPDB Arginase-1 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 2.1.4.1 false 2.1.4.1 Glycine + L-Arginine ↔ Guanidoacetic acid + Ornithine REVERSIBLE 2.1.1.2 false 2.1.1.2 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 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 2.6.1.13 false 2.6.1.13 Ornithine + Oxoglutaric acid ↔ L-Glutamic acid + L-Glutamic gamma-semialdehyde REVERSIBLE 1.5.5.2 false 1.5.5.2 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 1.5.1.2 false 1.5.1.2 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 1.5.5.2 false 1.5.5.2 1-Pyrroline-5-carboxylic acid ↔ L-Proline REVERSIBLE 1.5.1.2 false 1.5.1.2 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 2.1.3.3 false 2.1.3.3 Carbamoyl phosphate + Ornithine → Citrulline + Phosphate LEFT_TO_RIGHT C21H30N7O17P3 NADPH 745.0911 Mitochondrion C21H29N7O17P3 NADP 744.08325 C5H12N2O2 Ornithine 132.08987 6.1.1.19 false 6.1.1.19 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 Alanine Metabolism SubPathway Alanine Metabolism SubPathway D-Arginine and D-Ornithine Metabolism SubPathway D-Arginine and D-Ornithine Metabolism SubPathway 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 1.14.11.2 false 1.14.11.2 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) 6.3.4.16 false 6.3.4.16 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 Citric Acid Cycle SubPathway 3946848 PubChem-compound 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