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CHEBI:16015 ChEBI HMDB00134 HMDB 6022 PubChem-compound O75746 UniProt Q9UI32 UniProt 6262 PubChem-compound Citrulline SMP00029 SMPDB 937 ChemSpider 1.0 Ornithine carbamoyltransferase, mitochondrial HMDB00250 HMDB PW000183 PathWhiz CHEBI:16134 ChEBI HMDB01341 HMDB 2.0 L-Aspartic acid SubPathwayOutput 439153 PubChem-compound 110-17-8 CAS SMP00024 SMPDB Arginase-1 50 ChemSpider SMP00020 SMPDB C00327 KEGG Compound CHEBI:16027 ChEBI HMDB00148 HMDB 1.0 OXALACETIC_ACID BioCyc ATP BioCyc PW000052 PathWhiz Aspartate aminotransferase, mitochondrial 559142 ChemSpider SMP00036 SMPDB PW000046 PathWhiz PW000043 PathWhiz 5746 ChemSpider 5745 ChemSpider 5742 ChemSpider Argininemia Ornithine carbamoyltransferase, mitochondrial 1.0 L-ASPARTATE BioCyc 1143 ChemSpider PW000160 PathWhiz UREA BioCyc Transport139 false L-Glutamine (→) Transport: Homo sapiens, Extracellular Space to Homo sapiens, Cell, Cytoplasm LEFT_TO_RIGHT SMP00046 SMPDB 1.0 SMP00044 SMPDB HMDB00243 HMDB L-Glutamic acid ARG BioCyc 5858 ChemSpider Adenosine monophosphate Carbon dioxide Transport141 false L-Alanine (→) Transport: Homo sapiens, Extracellular Space to Homo sapiens, Cell, Cytoplasm LEFT_TO_RIGHT 5735 ChemSpider 1.0 L-CITRULLINE BioCyc PW000158 PathWhiz 945 ChemSpider PW000152 PathWhiz 16950 PubChem-compound PW000030 PathWhiz 1.0 SMP00050 SMPDB L-Aspartic acid PPI BioCyc SMP00057 SMPDB Arginase-1 1.0 SMP00055 SMPDB HMDB00214 HMDB C5H10N2O3 L-Glutamine 146.06914 CH4NO5P Carbamoyl phosphate 140.98271 Neutral amino acid transporter A L-Alanine SubPathwayInput Alanine aminotransferase 1 7664-38-2 CAS 1.0 HMDB00208 HMDB 6.0 1.0 C00009 KEGG Compound C00008 KEGG Compound HMDB00223 HMDB SMP00357 SMPDB L-ALPHA-ALANINE BioCyc C00001 KEGG Compound 1.0 C00122 KEGG Compound 56-41-7 CAS CHEBI:18361 ChEBI NADH C00004 KEGG Compound CHEBI:18367 ChEBI C00003 KEGG Compound 1032 ChemSpider 1031 ChemSpider C00002 KEGG Compound 1.0 Fumaric acid SubPathwayInput Carbamoyl-phosphate synthase [ammonia], mitochondrial HMDB01429 HMDB 3.5.3.1 false 3.5.3.1 L-Arginine + Water → Ornithine + Urea LEFT_TO_RIGHT SMP00009 SMPDB C6H13N3O3 Citrulline 175.09569 372-75-8 CAS C21H28N7O14P2 NAD 664.11694 1.0 C03406 KEGG Compound 1.0 SMP00127 SMPDB SMP00128 SMPDB 44367445 PubChem-compound SMP00129 SMPDB Adenosine triphosphate C00011 KEGG Compound 1.0 2.0 C00014 KEGG Compound Extracellular Space C00013 KEGG Compound Calcium-binding mitochondrial carrier protein Aralar1 Neutral amino acid transporter B(0) Phosphate C00022 KEGG Compound 56-65-5 CAS 53-84-9 CAS C00020 KEGG Compound C00026 KEGG Compound C00025 KEGG Compound CHEBI:26078 ChEBI SMP00015 SMPDB L-Glutamic acid SubPathwayOutput SubPathwayInput SubPathwayInteraction675 SubPathway675Reaction SubPathwayReaction SubPathwayInteraction676 SubPathway676Reaction SubPathwayReaction SubPathwayInteraction674 SubPathwayReaction SubPathway674Reaction SubPathwayInteraction679 SubPathway679Reaction SubPathwayReaction SubPathwayInteraction677 SubPathwayReaction SubPathway677Reaction 1.4.1.3 false 1.4.1.3 L-Glutamic acid + NAD + Water → Ammonia + NADH + Oxoglutaric acid LEFT_TO_RIGHT SubPathwayInteraction678 SubPathwayReaction SubPathway678Reaction CL:0000000 CELL TYPE ONTOLOGY 9367 ChemSpider C00036 KEGG Compound 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 1.0 C00041 KEGG Compound SMILES [O-]P([O-])(=O)OP([O-])([O-])=O Argininosuccinate synthase 1.0 L-Glutamine C00049 KEGG Compound 3.5.1.2 false 3.5.1.2 L-Glutamine + Water → Ammonia + L-Glutamic acid LEFT_TO_RIGHT C00169 KEGG Compound 56-85-9 CAS SMILES NC1=NC=NC2=C1N=CN2[C@@H]1O[C@H](COP(O)(=O)OP(O)(O)=O)[C@@H](O)[C@H]1O Alanine aminotransferase 1 Argininosuccinate lyase HMDB00538 HMDB CH4N2O Urea 60.032364 1.0 SubPathwayInteraction698 SubPathway698Reaction SubPathwayReaction 10197150 ChemSpider 4.3.2.1 false 4.3.2.1 2 Argininosuccinic acid → Fumaric acid + L-Arginine LEFT_TO_RIGHT SubPathwayInteraction699 SubPathway699Reaction SubPathwayReaction Ammonia SubPathwayOutput SubPathwayInput FUM BioCyc SMILES OC(=O)CC(=O)C(O)=O 7732-18-5 CAS 6.3.4.5 false 6.3.4.5 Adenosine triphosphate + Citrulline + L-Aspartic acid → Adenosine monophosphate + Argininosuccinic acid + Pyrophosphate LEFT_TO_RIGHT Aspartate aminotransferase, mitochondrial CHEBI:18012 ChEBI CHEBI:16199 ChEBI SMILES N[C@@H](CCCNC(N)=N)C(O)=O 61-19-8 CAS 1.0 SMILES NC1=C2N=CN([C@@H]3O[C@H](COP(O)(O)=O)[C@@H](O)[C@H]3O)C2=NC=N1 1.0 C00062 KEGG Compound SMILES N[C@@H](CCCNC(=N)N[C@@H](CC(O)=O)C(O)=O)C(O)=O Pyruvic acid AMMONIA BioCyc SMILES N 56-86-0 CAS 590-55-6 CAS Glutamate dehydrogenase 1, mitochondrial L-Aspartic acid HMDB00641 HMDB C10H16N5O13P3 Adenosine triphosphate 506.99576 CHEBI:17053 ChEBI C00064 KEGG Compound P24298 UniProt NAD 2387-71-5 CAS Pyrophosphate 2.6.1.2 false 2.6.1.2 L-Alanine + Oxoglutaric acid ↔ L-Glutamic acid + Pyruvic acid REVERSIBLE SMILES CC(=O)C(O)=O 1.0 HMDB01967 HMDB HMDB00517 HMDB 962 PubChem-compound Selenoamino Acid Metabolism SubPathway C3H7NO2 L-Alanine 89.047676 5800 ChemSpider 4.0 C00077 KEGG Compound CO2 Carbon dioxide 43.98983 979 ChemSpider Argininosuccinate synthase Carbamoyl-phosphate synthase [ammonia], mitochondrial 1.0 Water H3O4P Phosphoric acid 97.9769 BiologicalState8 Ammonia Recycling SubPathway SMILES N[C@@H](CCC(O)=O)C(O)=O BiologicalState3 Pyrimidine Metabolism SubPathway BiologicalState4 Glutamate Metabolism SubPathway Alanine Metabolism SubPathway Adenosine diphosphate Glucose-Alanine Cycle SubPathway L-Glutamine 14265-44-2 CAS PYRUVATE BioCyc 58-64-0 CAS Glutaminase liver isoform, mitochondrial Mitochondrial ornithine transporter 1 C00086 KEGG Compound Ornithine carbamoyltransferase, mitochondrial C4H7NO4 L-Aspartic acid 133.0375 644102 PubChem-compound L-Glutamine SubPathwayOutput SubPathwayInput 6083 PubChem-compound Argininosuccinate lyase Argininosuccinic acid CHEBI:18050 ChEBI H2O Water 18.010565 1.0 Phosphoric acid NADH BioCyc Malate-Aspartate Shuttle SubPathway Glutamate Metabolism SubPathway NAD BioCyc 0.0 Glutamate dehydrogenase 1, mitochondrial 56-84-8 CAS 1.0 Glutamate dehydrogenase 1, mitochondrial 970 PubChem-compound SMILES OC(=O)\C=C\C(O)=O P04424 UniProt 3.0 30572 ChemSpider SubPathwayInteraction712 SubPathway712Reaction SubPathwayReaction SubPathwayInteraction713 SubPathwayReaction SubPathway713Reaction C4H4O5 Oxalacetic acid 132.00587 ReactionCatalysis18 ACTIVATION SubPathwayInteraction710 SubPathway710Reaction SubPathwayReaction SubPathwayInteraction711 SubPathwayReaction SubPathway711Reaction SubPathwayInteraction716 SubPathwayReaction SubPathway716Reaction SubPathwayInteraction717 SubPathwayReaction SubPathway717Reaction Adenosine triphosphate SubPathwayInteraction714 SubPathway714Reaction SubPathwayReaction Argininosuccinate synthase SubPathwayInteraction715 SubPathway715Reaction SubPathwayReaction ReactionCatalysis12 ACTIVATION 1.0 SubPathwayInteraction718 SubPathway718Reaction SubPathwayReaction 21883788 PubChem-compound SMILES O C10H18N4O6 Argininosuccinic acid 290.12262 H3N Ammonia 17.026548 57-13-6 CAS C6H14N4O2 L-Arginine 174.11168 C10H14N5O7P Adenosine monophosphate 347.06308 4.0 P00480 UniProt 1.0 SubPathwayInteraction701 SubPathway701Reaction SubPathwayReaction 6992098 PubChem-compound SubPathwayInteraction702 SubPathwayReaction SubPathway702Reaction SubPathwayInteraction700 SubPathway700Reaction SubPathwayReaction SubPathwayInteraction705 SubPathwayReaction SubPathway705Reaction SubPathwayInteraction706 SubPathway706Reaction SubPathwayReaction 1004 PubChem-compound SubPathwayInteraction703 SubPathway703Reaction SubPathwayReaction SubPathwayInteraction704 SubPathwayReaction SubPathway704Reaction 5961 PubChem-compound SubPathwayInteraction709 SubPathwayReaction SubPathway709Reaction 1.0 SubPathwayInteraction707 SubPathway707Reaction SubPathwayReaction TransportCatalysis7 ACTIVATION SubPathwayInteraction708 SubPathwayReaction SubPathway708Reaction Ammonia 1.0 328-42-7 CAS 51 PubChem-compound Water CHEBI:15846 ChEBI P00367 UniProt ADP BioCyc C3H4O3 Pyruvic acid 88.016045 CHEBI:15729 ChEBI ReactionCatalysis137 ACTIVATION ReactionCatalysis38 ACTIVATION Neutral amino acid transporter B(0) 274 ChemSpider C5H6O5 Oxoglutaric acid 146.02153 272 ChemSpider HMDB00902 HMDB HMDB00904 HMDB Ornithine SubPathwayInput CHEBI:16908 ChEBI 70-26-8 CAS O4P Phosphate 94.95342 6026 ChemSpider Aspartate aminotransferase, mitochondrial ReactionCatalysis43 ACTIVATION Q9Y619 UniProt SMILES NC(N)=O 2.1.3.3 false 2.1.3.3 Carbamoyl phosphate + Ornithine → Citrulline + Phosphate LEFT_TO_RIGHT Mitochondrion TransportCatalysis103 ACTIVATION TransportCatalysis101 ACTIVATION C5H12N2O2 Ornithine 132.08987 Purine Metabolism SubPathway Glucose-Alanine Cycle SubPathway D-Arginine and D-Ornithine Metabolism SubPathway Reaction78 false L-Aspartic acid + Oxoglutaric acid ↔ L-Glutamic acid + Oxalacetic acid REVERSIBLE Arginine and Proline Metabolism SubPathway Citrulline D-Arginine and D-Ornithine Metabolism SubPathway Citric Acid Cycle SubPathway Aspartate Metabolism SubPathway CHEBI:16977 ChEBI 217 ChemSpider 58-68-4 CAS Water SMILES [O-]P([O-])([O-])=O SMILES OC(=O)CCC(=O)C(O)=O CHEBI:30915 ChEBI 124-38-9 CAS L-Alanine Glucose-Alanine Cycle SubPathway Ammonia Recycling SubPathway ReactionCatalysis2 ACTIVATION Arginase-1 SMILES NCCC[C@H](N)C(O)=O GO:0005758 GENE ONTOLOGY GO:0005759 GENE ONTOLOGY GLN BioCyc GLT BioCyc 7664-41-7 CAS C4H4O4 Fumaric acid 116.010956 SMILES O=C=O SMILES C[C@H](N)C(O)=O 2.0 5950 PubChem-compound 222 PubChem-compound 1.0 SMILES OP(O)(O)=O O7P2 Pyrophosphate 173.91193 C21H29N7O14P2 NADH 665.12476 6082 ChemSpider 1.0 5957 PubChem-compound L-Glutamic acid Alanine aminotransferase 1 2.0 HMDB01096 HMDB Ornithine GO:0005739 GENE ONTOLOGY GO:0005615 GENE ONTOLOGY C10H15N5O10P2 Adenosine diphosphate 427.02942 GO:0005737 GENE ONTOLOGY SMILES N[C@@H](CC(O)=O)C(O)=O 1.0 16059 ChemSpider 0.0 P05089 UniProt 2.0 SMILES N[C@@H](CCCNC(N)=O)C(O)=O TransportCatalysis12 ACTIVATION 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 CPD-8587 BioCyc 5682 ChemSpider CHEBI:15682 ChEBI Oxalacetic acid SubPathwayInput P43007 UniProt 1.0 Mitochondrial ornithine transporter 1 Mitochondrial Matrix Urea 14000-31-8 CAS CARBAMOYL-P BioCyc Neutral amino acid transporter A SMILES NC(=O)OP(O)(O)=O Cytoplasm Cell 1.0 1.0 CHEBI:15422 ChEBI 1.0 HMDB00191 HMDB 388299 ChemSpider 127-17-3 CAS Calcium-binding mitochondrial carrier protein Aralar1 28782 PubChem-compound C5H9NO4 L-Glutamic acid 147.05316 33032 PubChem-compound Glucose-Alanine Cycle SubPathway Gluconeogenesis SubPathway Histidine Metabolism SubPathway L-Arginine SubPathwayInput Porphyrin Metabolism SubPathway Transport39 false Ornithine (→), Citrulline (←) Transport: Homo sapiens, Cell, Cytoplasm to Homo sapiens, Cell, Mitochondrial Matrix LEFT_TO_RIGHT Alanine Metabolism SubPathway Ammonia Recycling SubPathway Glutathione Metabolism SubPathway CHEBI:16526 ChEBI Glutamate Metabolism SubPathway Glucose-Alanine Cycle SubPathway Transport31 false L-Aspartic acid (→), L-Glutamic acid (←) Transport: Homo sapiens, Cell, Mitochondrial Matrix to Homo sapiens, Cell, Mitochondrial Intermembrane Space LEFT_TO_RIGHT HMDB02142 HMDB CHEBI:16761 ChEBI Alanine Metabolism SubPathway 9606 TAXONOMY 2-KETOGLUTARATE BioCyc HMDB00051 HMDB Oxoglutaric acid SubPathwayOutput SubPathwayInput HMDB00294 HMDB HMDB00052 HMDB 5893 PubChem-compound 1176 PubChem-compound HMDB02111 HMDB L-Alanine SMP00067 SMPDB PW000019 PathWhiz Mitochondrial Intermembrane Space CHEBI:32816 ChEBI 1.0 CHEBI:15377 ChEBI 278 PubChem-compound CHEBI:16467 ChEBI PW000010 PathWhiz CHEBI:16349 ChEBI 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 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 P31327 UniProt 1.0 CHEBI:17672 ChEBI SMP00072 SMPDB 280 PubChem-compound CHEBI:30744 ChEBI PW000009 PathWhiz PW000007 PathWhiz Carbamoyl phosphate P00966 UniProt HMDB01487 HMDB PW000002 PathWhiz ReactionCatalysis136 ACTIVATION PW000001 PathWhiz ReactionCatalysis133 ACTIVATION PW000005 PathWhiz PW000004 PathWhiz ReactionCatalysis372 ACTIVATION PW000003 PathWhiz SMILES N[C@@H](CCC(N)=O)C(O)=O Q15758 UniProt 1060 PubChem-compound HMDB00045 HMDB 1061 PubChem-compound Glutaminase liver isoform, mitochondrial 74-79-3 CAS 1.0 HMDB00161 HMDB P00505 UniProt L-ORNITHINE BioCyc 3.0