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CHEBI:16015 ChEBI C00438 KEGG Compound 7722-84-1 CAS Citrulline SubPathwayOutput P30566 UniProt 937 ChemSpider Q8N142 UniProt 1.0 6267 PubChem-compound CHEBI:16134 ChEBI L-Aspartic acid SubPathwayInput HMDB0003125 HMDB 1.0 110-17-8 CAS D-aspartate oxidase 50 ChemSpider C00327 KEGG Compound CHEBI:16027 ChEBI OXALACETIC_ACID BioCyc ATP BioCyc beta-Alanine SubPathwayOutput SubPathwayInput 559142 ChemSpider 13184-27-5 CAS Hydrogen peroxide CHEBI:17202 ChEBI PW000043 PathWhiz 8582 PubChem-compound 5746 ChemSpider 1.0 5745 ChemSpider 5742 ChemSpider ASN BioCyc L-ASPARTATE BioCyc PW000162 PathWhiz PW000160 PathWhiz 952 ChemSpider Carbon dioxide Guanosine triphosphate L-Glutamic acid 5858 ChemSpider ARG BioCyc Adenosine monophosphate PW000032 PathWhiz L-CITRULLINE BioCyc PW000157 PathWhiz 1.0 CHEBI:16240 ChEBI 945 ChemSpider 16950 PubChem-compound PW000151 PathWhiz C00222 KEGG Compound Adenylosuccinate synthetase isozyme 1 C10H15N5O11P2 Guanosine diphosphate 443.02432 Asparagine synthetase [glutamine-hydrolyzing] PPI BioCyc Aspartate--tRNA ligase, cytoplasmic HMDB0001096 HMDB L-aspartyl-tRNA(Asp) 1.0 C10H16N5O14P3 Guanosine triphosphate 522.99066 C5H10N2O3 L-Glutamine 146.06914 Water CH4NO5P Carbamoyl phosphate 140.98271 1.0 L-Glutamic acid 1.0 Q99259 UniProt 2.0 C00009 KEGG Compound CAD protein 1.0 C00007 KEGG Compound PW000093 PathWhiz C00001 KEGG Compound C00122 KEGG Compound CHEBI:18361 ChEBI SMILES NC1=NC2=C(N=CN2[C@@H]2O[C@H](COP(O)(=O)OP(O)(O)=O)[C@@H](O)[C@H]2O)C(=O)N1 CHEBI:18367 ChEBI 1032 ChemSpider C00002 KEGG Compound Fumaric acid Malonic semialdehyde Adenylosuccinate lyase 93072 PubChem-compound C6H13N3O3 Citrulline 175.09569 372-75-8 CAS 1.0 C03406 KEGG Compound Hydrogen peroxide 44367445 PubChem-compound Adenosine triphosphate C00011 KEGG Compound C00130 KEGG Compound L-Asparagine HMDB0000904 HMDB C00014 KEGG Compound C00013 KEGG Compound CHEBI:17364 ChEBI 4-aminobutyrate aminotransferase, mitochondrial Q7L266 UniProt Ureidosuccinic acid 58576 ChemSpider 2.0 Carbamoyl phosphate 56-65-5 CAS C00020 KEGG Compound C00027 KEGG Compound C00026 KEGG Compound C00025 KEGG Compound Q99489 UniProt 1.0 ACET BioCyc Aspartoacylase 1.0 CHEBI:29172 ChEBI C00033 KEGG Compound C00152 KEGG Compound 9367 ChemSpider C00036 KEGG Compound Glutamate decarboxylase 1 C00035 KEGG Compound 328-50-7 CAS HMDB0000134 HMDB Argininosuccinate lyase AMP BioCyc Peroxisome 1.0 HYDROGEN-PEROXIDE BioCyc HMDB0000250 HMDB SMILES [O-]P([O-])(=O)OP([O-])([O-])=O Argininosuccinate synthase CHEBI:17196 ChEBI C00044 KEGG Compound L-Glutamine C00049 KEGG Compound C03794 KEGG Compound C00169 KEGG Compound 56-85-9 CAS Asparagine synthetase [glutamine-hydrolyzing] Adenylosuccinate lyase 763 ChemSpider HMDB0000148 HMDB Argininosuccinate lyase 6.0 C6H9NO5 N-Acetyl-L-aspartic acid 175.04807 1.4.3.2 false 1.4.3.2 L-Aspartic acid + Oxygen + Water ↔ Ammonia + Hydrogen peroxide + Oxalacetic acid REVERSIBLE Reaction14 false Carbamoyl phosphate + L-Aspartic acid ↔ Phosphate + Ureidosuccinic acid REVERSIBLE 6.3.4.4 false 6.3.4.4 Guanosine triphosphate + Inosinic acid + L-Aspartic acid ↔ Adenylsuccinic acid + Guanosine diphosphate + Phosphate REVERSIBLE 10197150 ChemSpider 784 PubChem-compound Oxoglutaric acid 6.1.1.12 false 6.1.1.12 Adenosine triphosphate + L-Aspartic acid + tRNA(Asp) ↔ Adenosine monophosphate + L-aspartyl-tRNA(Asp) + Pyrophosphate REVERSIBLE 1.4.3.1 false 1.4.3.1 D-Aspartic acid + Oxygen + Water ↔ Ammonia + Hydrogen peroxide + Oxalacetic acid REVERSIBLE CHEBI:21547 ChEBI 4.3.2.1 false 4.3.2.1 2 Argininosuccinic acid → Fumaric acid + L-Arginine LEFT_TO_RIGHT 1.0 4.3.2.2 false 4.3.2.2 Adenylsuccinic acid ↔ Adenosine monophosphate + Fumaric acid REVERSIBLE FUM BioCyc Oxalacetic acid SMILES CC(O)=O 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 6.1.1.22 false 6.1.1.22 Adenosine triphosphate + L-Asparagine + tRNA(Asn) ↔ Adenosine monophosphate + L-asparaginyl-tRNA(Asn) + Pyrophosphate REVERSIBLE CHEBI:18012 ChEBI 61-19-8 CAS SMILES N[C@@H](CCCNC(N)=N)C(O)=O SMILES NC1=C2N=CN([C@@H]3O[C@H](COP(O)(O)=O)[C@@H](O)[C@H]3O)C2=NC=N1 926-61-4 CAS SMILES N[C@@H](CCCNC(=N)N[C@@H](CC(O)=O)C(O)=O)C(O)=O C00062 KEGG Compound AMMONIA BioCyc SMILES N 56-86-0 CAS 590-55-6 CAS O43776 UniProt D-Aspartic acid CHEBI:29158 ChEBI C10H16N5O13P3 Adenosine triphosphate 506.99576 C14H18N5O11P Adenylsuccinic acid 463.07404 CHEBI:17053 ChEBI 6.3.5.4 false 6.3.5.4 Adenosine triphosphate + L-Aspartic acid + L-Glutamine + Water ↔ Adenosine monophosphate + L-Asparagine + L-Glutamic acid + Pyrophosphate REVERSIBLE 3.5.1.15 false 3.5.1.15 N-Acetyl-L-aspartic acid + Water ↔ Acetic acid + L-Aspartic acid REVERSIBLE 4.1.1.15 false 4.1.1.15 L-Aspartic acid → Carbon dioxide + beta-Alanine LEFT_TO_RIGHT C00064 KEGG Compound Asparagine--tRNA ligase, cytoplasmic Pyrophosphate 2387-71-5 CAS HMDB0001377 HMDB HMDB0000045 HMDB HMDB0000168 HMDB Aspartate--tRNA ligase, cytoplasmic C01042 KEGG Compound Reaction5 false Oxoglutaric acid + beta-Alanine → L-Glutamic acid + Malonic semialdehyde LEFT_TO_RIGHT C5H8N2O5 Ureidosuccinic acid 176.04332 SMILES NCCC(O)=O HMDB0000042 HMDB CHEBI:29265 ChEBI 962 PubChem-compound 4.0 C4H7NO4 D-Aspartic acid 133.0375 CO2 Carbon dioxide 43.98983 1.0 Argininosuccinate synthase HMDB0000056 HMDB Ammonia SMILES O=O SMILES OC(=O)CC=O HMDB0000051 HMDB C4H8N2O3 L-Asparagine 132.0535 SMILES N[C@@H](CCC(O)=O)C(O)=O HMDB0002111 HMDB HMDB0000052 HMDB CHEBI:15919 ChEBI HMDB0000175 HMDB 14265-44-2 CAS C10H13N4O8P Inosinic acid 348.0471 1.0 4.0 845 ChemSpider GTP BioCyc 2.0 C4H7NO4 L-Aspartic acid 133.0375 HMDB0001273 HMDB Inosinic acid 644102 PubChem-compound 868 PubChem-compound 6083 PubChem-compound Argininosuccinate lyase C00099 KEGG Compound Aspartoacylase 1.0 1.0 1.0 Argininosuccinic acid CHEBI:18050 ChEBI H2O Water 18.010565 H2O2 Hydrogen peroxide 34.005478 Oxygen 1.0 HMDB0000191 HMDB 1.0 Ammonia 1.0 977 PubChem-compound Adenylosuccinate lyase 56-84-8 CAS 970 PubChem-compound 1.0 SMILES OC(=O)\C=C\C(O)=O 70-47-3 CAS 146-91-8 CAS P04424 UniProt CHEBI:29186 ChEBI 1.0 ReactionCatalysis16 ACTIVATION ReactionCatalysis17 ACTIVATION 30572 ChemSpider ReactionCatalysis14 ACTIVATION ReactionCatalysis15 ACTIVATION C2H4O2 Acetic acid 60.02113 Adenylosuccinate synthetase isozyme 1 Q96RQ9 UniProt ReactionCatalysis18 ACTIVATION C4H4O5 Oxalacetic acid 132.00587 ReactionCatalysis19 ACTIVATION P45381 UniProt Argininosuccinate synthase ReactionCatalysis12 ACTIVATION ReactionCatalysis13 ACTIVATION ReactionCatalysis10 ACTIVATION ReactionCatalysis11 ACTIVATION 64-19-7 CAS HMDB0000536 HMDB 21883788 PubChem-compound SMILES O SMILES OO C10H18N4O6 Argininosuccinic acid 290.12262 H3N Ammonia 17.026548 C6H14N4O2 L-Arginine 174.11168 C10H14N5O7P Adenosine monophosphate 347.06308 4.0 1.0 6992098 PubChem-compound CPD-302 BioCyc L-amino-acid oxidase CPD-420 BioCyc 239 PubChem-compound HMDB0000538 HMDB 5961 PubChem-compound 1.0 1.0 P27708 UniProt Histidine Metabolism SubPathway 328-42-7 CAS 51 PubChem-compound Water 131-99-7 CAS Nicotinate and Nicotinamide Metabolism SubPathway C3H7NO2 beta-Alanine 89.047676 Pyrimidine Metabolism SubPathway SubPathwayInteraction851 SubPathway851Reaction SubPathwayReaction SubPathwayInteraction852 SubPathwayReaction SubPathway852Reaction SubPathwayInteraction850 SubPathway850Reaction SubPathwayReaction Lysosome SubPathwayInteraction853 SubPathwayReaction SubPathway853Reaction 1.0 SubPathwayInteraction854 SubPathway854Reaction SubPathwayReaction Glutamate decarboxylase 1 Canavan Disease 1.0 274 ChemSpider C5H6O5 Oxoglutaric acid 146.02153 beta-Alanine 272 ChemSpider Urea Cycle SubPathway 1.0 O4P Phosphate 94.95342 SubPathwayInteraction848 SubPathway848Reaction SubPathwayReaction SubPathwayInteraction849 SubPathwayReaction SubPathway849Reaction SubPathwayInteraction846 SubPathwayReaction SubPathway846Reaction SubPathwayInteraction847 SubPathwayReaction SubPathway847Reaction 6830 PubChem-compound 6031 ChemSpider Asparagine--tRNA ligase, cytoplasmic Mitochondrion ReactionCatalysis2200 ACTIVATION L-Asparagine HMDB0000208 HMDB CPD-6641 BioCyc 217 ChemSpider 1.0 SMILES [O-]P([O-])([O-])=O SMILES OC(=O)CCC(=O)C(O)=O CHEBI:30915 ChEBI ReactionCatalysis8 ACTIVATION 124-38-9 CAS SMP0000048 SMPDB ReactionCatalysis6 ACTIVATION GO:0005764 GENE ONTOLOGY SMP0000046 SMPDB SMP0000044 SMPDB ReactionCatalysis9 ACTIVATION Adenylsuccinic acid CAD protein HMDB0001429 HMDB 19046-78-7 CAS HMDB0011111 HMDB HMDB0000223 HMDB GDP-4-DEHYDRO-6-DEOXY-D-MANNOSE BioCyc GLN BioCyc GLT BioCyc 7664-41-7 CAS SMP0000059 SMPDB 107-95-9 CAS beta-Alanine Metabolism SubPathway 84022 ChemSpider C4H4O4 Fumaric acid 116.010956 SMP0000175 SMPDB Glycine and Serine Metabolism SubPathway Oxalacetic acid Isoaspartyl peptidase/L-asparaginase SMILES O=C=O SMILES N[C@H](CC(O)=O)C(O)=O 2.0 222 PubChem-compound SMILES N[C@@H](CC(N)=O)C(O)=O 8264 ChemSpider HMDB0001201 HMDB O7P2 Pyrophosphate 173.91193 234 ChemSpider SMILES O[C@@H]1[C@@H](COP(O)(O)=O)O[C@H]([C@@H]1O)N1C=NC2=C1N=CNC2=O 6082 ChemSpider 1.0 5957 PubChem-compound Aspartoacylase CHEBI:15859 ChEBI Adenylosuccinate synthetase isozyme 1 CHEBI:15996 ChEBI 75697 ChemSpider CARBAMYUL-L-ASPARTATE BioCyc P14868 UniProt GO:0005739 GENE ONTOLOGY Arginine and Proline Metabolism SubPathway GO:0005737 GENE ONTOLOGY SMILES N[C@@H](CC(O)=O)C(O)=O CHEBI:16958 ChEBI 16059 ChemSpider Pantothenate and CoA Biosynthesis SubPathway 2.0 SMP0000007 SMPDB SMILES N[C@@H](CCCNC(N)=O)C(O)=O CPD-8587 BioCyc 2.0 1.0 CHEBI:15682 ChEBI 1.0 1.0 1.0 1.0 SMP0000004 SMPDB IMP BioCyc CAD protein 14000-31-8 CAS CARBAMOYL-P BioCyc Glutamate decarboxylase 1 HMDB0000812 HMDB 65065 PubChem-compound B-ALANINE BioCyc P08243 UniProt 1.0 Oxygen SMILES NC(=O)OP(O)(O)=O 1.0 Cytoplasm HMDB0006483 HMDB 1.0 CHEBI:15422 ChEBI 4-aminobutyrate aminotransferase, mitochondrial SMP0000027 SMPDB P80404 UniProt 997-55-7 CAS Homo sapiens HMDB0000828 HMDB CHEBI:17960 ChEBI Guanosine diphosphate C3H4O3 Malonic semialdehyde 88.016045 O2 Oxygen 31.98983 28782 PubChem-compound C5H9NO4 L-Glutamic acid 147.05316 SMP0000020 SMPDB 33032 PubChem-compound Aspartate--tRNA ligase, cytoplasmic Water L-Arginine CHEBI:16526 ChEBI 8977 PubChem-compound tRNA(Asn) 7782-44-7 CAS GO:0005777 GENE ONTOLOGY 1783-96-6 CAS L-Aspartic acid 9606 TAXONOMY CHEBI:15366 ChEBI 2-KETOGLUTARATE BioCyc SMILES CC(=O)N[C@@H](CC(O)=O)C(O)=O 8630 ChemSpider 389122 ChemSpider Reaction2307 false L-Asparagine + Water → Ammonia + L-Aspartic acid LEFT_TO_RIGHT tRNA(Asp) 171 ChemSpider 1.0 CHEBI:15377 ChEBI 278 PubChem-compound CHEBI:15379 ChEBI PW000011 PathWhiz CHEBI:16467 ChEBI C00402 KEGG Compound PW000010 PathWhiz CHEBI:16349 ChEBI 6569 ChemSpider 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 SMILES O[C@@H]1[C@@H](COP(O)(O)=O)O[C@H]([C@@H]1O)N1C=NC2=C1N=CN=C2NC(CC(O)=O)C(O)=O CHEBI:17552 ChEBI 1.0 Phosphate CHEBI:17672 ChEBI 280 PubChem-compound SMILES NC(=O)N[C@@H](CC(O)=O)C(O)=O CHEBI:30744 ChEBI N-Acetyl-L-aspartic acid P00966 UniProt L-asparaginyl-tRNA(Asn) tRNA(Asn) L-aspartyl-tRNA(Asp) tRNA(Asp) L-asparaginyl-tRNA(Asn) SMILES NC1=NC2=C(N=CN2[C@@H]2O[C@H](COP(O)(=O)OP(O)(=O)OP(O)(O)=O)[C@@H](O)[C@H]2O)C(=O)N1 Acetic acid SMILES N[C@@H](CCC(N)=O)C(O)=O 440122 PubChem-compound L-amino-acid oxidase 1.0 83887 PubChem-compound 86-01-1 CAS 1061 PubChem-compound D-aspartate oxidase 74-79-3 CAS HMDB0001967 HMDB HMDB0000517 HMDB 176 PubChem-compound HMDB0000641 HMDB 1.0 4-aminobutyrate aminotransferase, mitochondrial 1.0 Isoaspartyl peptidase/L-asparaginase