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CHEBI:16015 ChEBI 6022 PubChem-compound O75746 UniProt Q9UI32 UniProt 6262 PubChem-compound Citrulline 937 ChemSpider 1.0 Ornithine carbamoyltransferase, mitochondrial CHEBI:16134 ChEBI 2.0 L-Aspartic acid SubPathwayOutput 439153 PubChem-compound 110-17-8 CAS Arginase-1 50 ChemSpider C00327 KEGG Compound CHEBI:16027 ChEBI 1.0 OXALACETIC_ACID BioCyc ATP BioCyc PW000052 PathWhiz Aspartate aminotransferase, mitochondrial 559142 ChemSpider PW000046 PathWhiz PW000043 PathWhiz 5746 ChemSpider 5745 ChemSpider 5742 ChemSpider Ornithine carbamoyltransferase, mitochondrial PW000162 PathWhiz 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 1.0 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 L-Aspartic acid PPI BioCyc HMDB0001096 HMDB Arginase-1 1.0 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 6.0 1.0 C00009 KEGG Compound C00008 KEGG Compound 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 3.5.3.1 false 3.5.3.1 L-Arginine + Water → Ornithine + Urea LEFT_TO_RIGHT C6H13N3O3 Citrulline 175.09569 372-75-8 CAS C21H28N7O14P2 NAD 664.11694 1.0 C03406 KEGG Compound 1.0 44367445 PubChem-compound Adenosine triphosphate C00011 KEGG Compound 1.0 HMDB0000902 HMDB 2.0 HMDB0000904 HMDB 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 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 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 HMDB0000134 HMDB Argininosuccinate lyase AMP BioCyc 1.0 C00041 KEGG Compound HMDB0000250 HMDB SMILES [O-]P([O-])(=O)OP([O-])([O-])=O HMDB0001341 HMDB 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 HMDB0000148 HMDB Argininosuccinate lyase 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 HMDB0001487 HMDB 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 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 HMDB0000045 HMDB 1.0 HMDB0000161 HMDB 962 PubChem-compound 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 Glutamate Metabolism SubPathway HMDB0000051 HMDB SMILES N[C@@H](CCC(O)=O)C(O)=O HMDB0002111 HMDB HMDB0000294 HMDB HMDB0000052 HMDB Adenosine diphosphate 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 HMDB0000191 HMDB NADH BioCyc 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 HMDB0002142 HMDB 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 D-Arginine and D-Ornithine Metabolism SubPathway 4.0 P00480 UniProt 1.0 SubPathwayInteraction701 SubPathway701Reaction SubPathwayReaction 6992098 PubChem-compound SubPathwayInteraction702 SubPathwayReaction SubPathway702Reaction SubPathwayInteraction700 SubPathway700Reaction SubPathwayReaction HMDB0000538 HMDB SubPathwayInteraction705 SubPathwayReaction SubPathway705Reaction SubPathwayInteraction706 SubPathway706Reaction SubPathwayReaction 1004 PubChem-compound SubPathwayInteraction703 SubPathway703Reaction SubPathwayReaction SubPathwayInteraction704 SubPathwayReaction SubPathway704Reaction 5961 PubChem-compound SubPathwayInteraction709 SubPathwayReaction SubPathway709Reaction 1.0 TransportCatalysis7 ACTIVATION SubPathwayInteraction708 SubPathwayReaction SubPathway708Reaction Histidine Metabolism SubPathway 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 Pyrimidine Metabolism SubPathway ReactionCatalysis38 ACTIVATION Neutral amino acid transporter B(0) Alanine Metabolism SubPathway Purine Metabolism SubPathway 274 ChemSpider C5H6O5 Oxoglutaric acid 146.02153 272 ChemSpider Ornithine SubPathwayInput Urea Cycle CHEBI:16908 ChEBI Citric Acid Cycle SubPathway 70-26-8 CAS O4P Phosphate 94.95342 6026 ChemSpider Aspartate aminotransferase, mitochondrial ReactionCatalysis43 ACTIVATION Aspartate Metabolism SubPathway 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 Reaction78 false L-Aspartic acid + Oxoglutaric acid ↔ L-Glutamic acid + Oxalacetic acid REVERSIBLE Citrulline CHEBI:16977 ChEBI HMDB0000208 HMDB 217 ChemSpider 58-68-4 CAS Water SMILES [O-]P([O-])([O-])=O HMDB0000214 HMDB SMILES OC(=O)CCC(=O)C(O)=O CHEBI:30915 ChEBI 124-38-9 CAS L-Alanine SMP0000046 SMPDB SMP0000044 SMPDB ReactionCatalysis2 ACTIVATION HMDB0001429 HMDB Arginase-1 SMP0000050 SMPDB HMDB0000223 HMDB SMILES NCCC[C@H](N)C(O)=O GO:0005758 GENE ONTOLOGY GO:0005759 GENE ONTOLOGY GLN BioCyc Ammonia Recycling SubPathway SMP0000059 SMPDB GLT BioCyc 7664-41-7 CAS Malate-Aspartate Shuttle SubPathway Gluconeogenesis SubPathway C4H4O4 Fumaric acid 116.010956 Glucose-Alanine Cycle SubPathway SMP0000057 SMPDB SMP0000055 SMPDB 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 SMP0000067 SMPDB L-Glutamic acid Alanine aminotransferase 1 2.0 Ornithine SMP0000072 SMPDB HMDB0000243 HMDB GO:0005739 GENE ONTOLOGY Arginine and Proline Metabolism SubPathway 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 Selenoamino Acid Metabolism SubPathway 16059 ChemSpider Porphyrin Metabolism SubPathway 0.0 P05089 UniProt SMP0000009 SMPDB 2.0 SMP0000128 SMPDB SMP0000129 SMPDB SMP0000127 SMPDB 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 1.0 1.0 CHEBI:15422 ChEBI SMP0000029 SMPDB 1.0 Homo sapiens 388299 ChemSpider 127-17-3 CAS Calcium-binding mitochondrial carrier protein Aralar1 28782 PubChem-compound SMP0000024 SMPDB C5H9NO4 L-Glutamic acid 147.05316 SMP0000020 SMPDB 33032 PubChem-compound L-Arginine SubPathwayInput Transport39 false Ornithine (→), Citrulline (←) Transport: Homo sapiens, Cell, Cytoplasm to Homo sapiens, Cell, Mitochondrial Matrix LEFT_TO_RIGHT CHEBI:16526 ChEBI Transport31 false L-Aspartic acid (→), L-Glutamic acid (←) Transport: Homo sapiens, Cell, Mitochondrial Matrix to Homo sapiens, Cell, Mitochondrial Intermembrane Space LEFT_TO_RIGHT CHEBI:16761 ChEBI SMP0000036 SMPDB 9606 TAXONOMY 2-KETOGLUTARATE BioCyc Oxoglutaric acid SubPathwayOutput SubPathwayInput 5893 PubChem-compound 1176 PubChem-compound L-Alanine 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 280 PubChem-compound CHEBI:30744 ChEBI PW000009 PathWhiz PW000007 PathWhiz Carbamoyl phosphate P00966 UniProt PW000002 PathWhiz ReactionCatalysis136 ACTIVATION PW000001 PathWhiz ReactionCatalysis133 ACTIVATION PW000005 PathWhiz ReactionCatalysis372 ACTIVATION PW000003 PathWhiz SMILES N[C@@H](CCC(N)=O)C(O)=O Q15758 UniProt 1060 PubChem-compound 1061 PubChem-compound Glutaminase liver isoform, mitochondrial 74-79-3 CAS HMDB0001967 HMDB 1.0 HMDB0000517 HMDB P00505 UniProt HMDB0000641 HMDB L-ORNITHINE BioCyc 3.0 Neutral amino acid transporter B(0) Neutral amino acid transporter A Mitochondrial ornithine transporter 1 Mitochondrial ornithine transporter 1 Calcium-binding mitochondrial carrier protein Aralar1 Alanine aminotransferase 1 Glutaminase liver isoform, mitochondrial Glutamate dehydrogenase 1, mitochondrial Carbamoyl- phosphate synthase [ammonia], mitochondrial Aspartate aminotransferase, mitochondrial Ornithine carbamoyltransferase, mitochondrial Arginase-1 Argininosuccinate lyase Argininosuccinate synthase L-Glutamine L-Alanine Oxoglutaric acid L-Glutamic acid Oxalacetic acid NH 3 L-Aspartic acid Fumaric acid L-Arginine Ornithine L-Glutamine L-Alanine Ornithine Citrulline Citrulline Ornithine Ornithine Citrulline Citrulline L-Aspartic acid L-Glutamic acid L-Glutamic acid Pyruvic acid H 2 O H 2 O NAD Oxoglutaric acid NADH ATP CO 2 H 2 O ADP P i Carbamoyl phosphate P i H 2 O Urea Argininosuccinic acid ATP AMP PP i Calcium Pyridoxal 5'-phosphate Pyridoxal 5'-phosphate Manganese Ammonia Recycling Pyrimidine Metabolism Glutamate Metabolism Alanine Metabolism Glucose-Alanine Cycle Selenoamino Acid Metabolism Malate- Aspartate Shuttle Glutamate Metabolism Alanine Metabolism Glucose-Alanine Cycle Gluconeogenesis Histidine Metabolism Porphyrin Metabolism Glucose-Alanine Cycle Porphyrin Metabolism Alanine Metabolism Ammonia Recycling Glutamate Metabolism Glucose-Alanine Cycle Ammonia Recycling Purine Metabolism Glucose-Alanine Cycle Aspartate Metabolism Citric Acid Cycle Arginine and Proline Metabolism D-Arginine and D-Ornithine Metabolism D-Arginine and D-Ornithine Metabolism Cytosol Mitochondria Mitochondrial Matrix Urea Cycle Extracellular Space Intracellular Space
CHEBI:16015 ChEBI 6022 PubChem-compound O75746 UniProt Q9UI32 UniProt 6262 PubChem-compound Citrulline 937 ChemSpider 1.0 Ornithine carbamoyltransferase, mitochondrial CHEBI:16134 ChEBI 2.0 L-Aspartic acid SubPathwayOutput 439153 PubChem-compound 110-17-8 CAS Arginase-1 50 ChemSpider C00327 KEGG Compound CHEBI:16027 ChEBI 1.0 OXALACETIC_ACID BioCyc ATP BioCyc PW000052 PathWhiz Aspartate aminotransferase, mitochondrial 559142 ChemSpider PW000046 PathWhiz PW000043 PathWhiz 5746 ChemSpider 5745 ChemSpider 5742 ChemSpider Ornithine carbamoyltransferase, mitochondrial PW000162 PathWhiz 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 1.0 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 L-Aspartic acid PPI BioCyc HMDB0001096 HMDB Arginase-1 1.0 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 6.0 1.0 C00009 KEGG Compound C00008 KEGG Compound 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 3.5.3.1 false 3.5.3.1 L-Arginine + Water → Ornithine + Urea LEFT_TO_RIGHT C6H13N3O3 Citrulline 175.09569 372-75-8 CAS C21H28N7O14P2 NAD 664.11694 1.0 C03406 KEGG Compound 1.0 44367445 PubChem-compound Adenosine triphosphate C00011 KEGG Compound 1.0 HMDB0000902 HMDB 2.0 HMDB0000904 HMDB 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 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 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 HMDB0000134 HMDB Argininosuccinate lyase AMP BioCyc 1.0 C00041 KEGG Compound HMDB0000250 HMDB SMILES [O-]P([O-])(=O)OP([O-])([O-])=O HMDB0001341 HMDB 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 HMDB0000148 HMDB Argininosuccinate lyase 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 HMDB0001487 HMDB 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 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 HMDB0000045 HMDB 1.0 HMDB0000161 HMDB 962 PubChem-compound 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 Glutamate Metabolism SubPathway HMDB0000051 HMDB SMILES N[C@@H](CCC(O)=O)C(O)=O HMDB0002111 HMDB HMDB0000294 HMDB HMDB0000052 HMDB Adenosine diphosphate 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 HMDB0000191 HMDB NADH BioCyc 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 HMDB0002142 HMDB 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 D-Arginine and D-Ornithine Metabolism SubPathway 4.0 P00480 UniProt 1.0 SubPathwayInteraction701 SubPathway701Reaction SubPathwayReaction 6992098 PubChem-compound SubPathwayInteraction702 SubPathwayReaction SubPathway702Reaction SubPathwayInteraction700 SubPathway700Reaction SubPathwayReaction HMDB0000538 HMDB SubPathwayInteraction705 SubPathwayReaction SubPathway705Reaction SubPathwayInteraction706 SubPathway706Reaction SubPathwayReaction 1004 PubChem-compound SubPathwayInteraction703 SubPathway703Reaction SubPathwayReaction SubPathwayInteraction704 SubPathwayReaction SubPathway704Reaction 5961 PubChem-compound SubPathwayInteraction709 SubPathwayReaction SubPathway709Reaction 1.0 TransportCatalysis7 ACTIVATION SubPathwayInteraction708 SubPathwayReaction SubPathway708Reaction Histidine Metabolism SubPathway 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 Pyrimidine Metabolism SubPathway ReactionCatalysis38 ACTIVATION Neutral amino acid transporter B(0) Alanine Metabolism SubPathway Purine Metabolism SubPathway 274 ChemSpider C5H6O5 Oxoglutaric acid 146.02153 272 ChemSpider Ornithine SubPathwayInput Urea Cycle CHEBI:16908 ChEBI Citric Acid Cycle SubPathway 70-26-8 CAS O4P Phosphate 94.95342 6026 ChemSpider Aspartate aminotransferase, mitochondrial ReactionCatalysis43 ACTIVATION Aspartate Metabolism SubPathway 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 Reaction78 false L-Aspartic acid + Oxoglutaric acid ↔ L-Glutamic acid + Oxalacetic acid REVERSIBLE Citrulline CHEBI:16977 ChEBI HMDB0000208 HMDB 217 ChemSpider 58-68-4 CAS Water SMILES [O-]P([O-])([O-])=O HMDB0000214 HMDB SMILES OC(=O)CCC(=O)C(O)=O CHEBI:30915 ChEBI 124-38-9 CAS L-Alanine SMP0000046 SMPDB SMP0000044 SMPDB ReactionCatalysis2 ACTIVATION HMDB0001429 HMDB Arginase-1 SMP0000050 SMPDB HMDB0000223 HMDB SMILES NCCC[C@H](N)C(O)=O GO:0005758 GENE ONTOLOGY GO:0005759 GENE ONTOLOGY GLN BioCyc Ammonia Recycling SubPathway SMP0000059 SMPDB GLT BioCyc 7664-41-7 CAS Malate-Aspartate Shuttle SubPathway Gluconeogenesis SubPathway C4H4O4 Fumaric acid 116.010956 Glucose-Alanine Cycle SubPathway SMP0000057 SMPDB SMP0000055 SMPDB 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 SMP0000067 SMPDB L-Glutamic acid Alanine aminotransferase 1 2.0 Ornithine SMP0000072 SMPDB HMDB0000243 HMDB GO:0005739 GENE ONTOLOGY Arginine and Proline Metabolism SubPathway 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 Selenoamino Acid Metabolism SubPathway 16059 ChemSpider Porphyrin Metabolism SubPathway 0.0 P05089 UniProt SMP0000009 SMPDB 2.0 SMP0000128 SMPDB SMP0000129 SMPDB SMP0000127 SMPDB 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 1.0 1.0 CHEBI:15422 ChEBI SMP0000029 SMPDB 1.0 Homo sapiens 388299 ChemSpider 127-17-3 CAS Calcium-binding mitochondrial carrier protein Aralar1 28782 PubChem-compound SMP0000024 SMPDB C5H9NO4 L-Glutamic acid 147.05316 SMP0000020 SMPDB 33032 PubChem-compound L-Arginine SubPathwayInput Transport39 false Ornithine (→), Citrulline (←) Transport: Homo sapiens, Cell, Cytoplasm to Homo sapiens, Cell, Mitochondrial Matrix LEFT_TO_RIGHT CHEBI:16526 ChEBI Transport31 false L-Aspartic acid (→), L-Glutamic acid (←) Transport: Homo sapiens, Cell, Mitochondrial Matrix to Homo sapiens, Cell, Mitochondrial Intermembrane Space LEFT_TO_RIGHT CHEBI:16761 ChEBI SMP0000036 SMPDB 9606 TAXONOMY 2-KETOGLUTARATE BioCyc Oxoglutaric acid SubPathwayOutput SubPathwayInput 5893 PubChem-compound 1176 PubChem-compound L-Alanine 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 280 PubChem-compound CHEBI:30744 ChEBI PW000009 PathWhiz PW000007 PathWhiz Carbamoyl phosphate P00966 UniProt PW000002 PathWhiz ReactionCatalysis136 ACTIVATION PW000001 PathWhiz ReactionCatalysis133 ACTIVATION PW000005 PathWhiz ReactionCatalysis372 ACTIVATION PW000003 PathWhiz SMILES N[C@@H](CCC(N)=O)C(O)=O Q15758 UniProt 1060 PubChem-compound 1061 PubChem-compound Glutaminase liver isoform, mitochondrial 74-79-3 CAS HMDB0001967 HMDB 1.0 HMDB0000517 HMDB P00505 UniProt HMDB0000641 HMDB L-ORNITHINE BioCyc 3.0