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CHEBI:16015 ChEBI 6022 PubChem-compound Q9UI32 UniProt 937 ChemSpider 1.0 HMDB00250 HMDB Aminomethyltransferase, mitochondrial CHEBI:16134 ChEBI L-Histidine 6267 PubChem-compound HMDB01341 HMDB CHEBI:30314 ChEBI 1.0 L-Aspartic acid 2.0 439153 PubChem-compound 50 ChemSpider CHEBI:20506 ChEBI 53481912 PubChem-compound CHEBI:17115 ChEBI CHEBI:16027 ChEBI HMDB00148 HMDB ATP BioCyc 18714427 ChemSpider 559142 ChemSpider Tetrahydrofolic acid Glycine dehydrogenase [decarboxylating], mitochondrial PW000043 PathWhiz 5746 ChemSpider P09622 UniProt 5745 ChemSpider ASN BioCyc 5742 ChemSpider L-ASPARTATE BioCyc 1.0 PW000162 PathWhiz 1.0 439175 PubChem-compound 1.0 SMP00044 SMPDB HMDB00243 HMDB 5858 ChemSpider L-Glutamic acid SubPathwayInput 5736 ChemSpider HMDB00123 HMDB 6274 PubChem-compound Adenosine monophosphate Carbon dioxide PW000157 PathWhiz METHYLENE-THF BioCyc C00101 KEGG Compound HMDB01451 HMDB HMDB12210 HMDB SMILES OC(=O)CCCCC(S)CCS Asparagine synthetase [glutamine-hydrolyzing] PPI BioCyc SMP00057 SMPDB SMP00059 SMPDB 643824 ChemSpider 1.0 5886 ChemSpider C5H10N2O3 L-Glutamine 146.06914 CH4NO5P Carbamoyl phosphate 140.98271 L-Glutamic acid Cytosol 7664-38-2 CAS 1.0 HMDB00208 HMDB Urocanic acid 6.0 1.0 C00009 KEGG Compound C00008 KEGG Compound C00001 KEGG Compound 1.0 HMDB13639 HMDB CHEBI:18361 ChEBI NADH CHEBI:18367 ChEBI C00004 KEGG Compound 1032 ChemSpider C00003 KEGG Compound 135-16-0 CAS 1031 ChemSpider C00002 KEGG Compound 388320 ChemSpider Carbamoyl-phosphate synthase [ammonia], mitochondrial HMDB01429 HMDB Reaction168 false L-Asparagine + Water ↔ Ammonia + L-Aspartic acid REVERSIBLE SMP00009 SMPDB Carbamoyl phosphate SubPathwayInput Pyruvic acid SubPathwayInput 1.0 1.0 C21H28N7O14P2 NAD 664.11694 44367445 PubChem-compound Adenosine triphosphate C00011 KEGG Compound L-Asparagine 2.0 C00014 KEGG Compound C00135 KEGG Compound C00013 KEGG Compound A8YXX4 UniProt Q7L266 UniProt 1.0 SMP00004 SMPDB Pyruvic acid 6112 PubChem-compound HMDB01533 HMDB Phosphate C00022 KEGG Compound C00143 KEGG Compound 56-65-5 CAS C00020 KEGG Compound 53-84-9 CAS C00026 KEGG Compound C00025 KEGG Compound CHEBI:26078 ChEBI SMILES NCSCCC(S)CCCCC(O)=O 3432-99-3 CAS 104-98-3 CAS L-Glutamic acid 6.3.1.2 false 6.3.1.2 Adenosine triphosphate + Ammonia + L-Glutamic acid → Adenosine diphosphate + L-Glutamine + Phosphate LEFT_TO_RIGHT 1.4.1.3 false 1.4.1.3 L-Glutamic acid + NAD + Water → Ammonia + NADH + Oxoglutaric acid LEFT_TO_RIGHT CL:0000000 CELL TYPE ONTOLOGY C00152 KEGG Compound 1.0 C00037 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 L-Histidine SubPathwayOutput AMP BioCyc Transport7 false Ammonia (→) Transport: Homo sapiens, Cell, Cytosol to Homo sapiens, Cell, Mitochondrial Matrix LEFT_TO_RIGHT SMILES [O-]P([O-])(=O)OP([O-])([O-])=O HMDB00301 HMDB 1.0 LIPOIC-ACID BioCyc CHEBI:17196 ChEBI 408 ChemSpider L-Glutamine C00049 KEGG Compound CHEBI:18047 ChEBI C00169 KEGG Compound 3.5.1.2 false 3.5.1.2 L-Glutamine + Water → Ammonia + L-Glutamic acid LEFT_TO_RIGHT 10.0 56-85-9 CAS Asparagine synthetase [glutamine-hydrolyzing] SMILES NC1=NC=NC2=C1N=CN2[C@@H]1O[C@H](COP(O)(=O)OP(O)(O)=O)[C@@H](O)[C@H]1O (R)-lipoic acid HMDB00538 HMDB 421 PubChem-compound Ammonia 7732-18-5 CAS 61-19-8 CAS SMILES NC1=C2N=CN([C@@H]3O[C@H](COP(O)(O)=O)[C@@H](O)[C@H]3O)C2=NC=N1 SMILES OC(=O)CCCC[C@@H]1CCSS1 1.0 AMMONIA BioCyc SMILES N 56-86-0 CAS 590-55-6 CAS Glutamate dehydrogenase 1, mitochondrial HMDB00641 HMDB 1.0 C10H16N5O13P3 Adenosine triphosphate 506.99576 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 56-40-6 CAS C00065 KEGG Compound C00064 KEGG Compound NAD Pyrophosphate SMILES CC(=O)C(O)=O 1.0 Ammonia HMDB01967 HMDB HMDB01846 HMDB UROCANATE BioCyc 962 PubChem-compound 5800 ChemSpider CO2 Carbon dioxide 43.98983 979 ChemSpider Carbamoyl-phosphate synthase [ammonia], mitochondrial 730 ChemSpider Water Glycine dehydrogenase [decarboxylating], mitochondrial Histidine ammonia-lyase H3O4P Phosphoric acid 97.9769 BiologicalState8 C4H8N2O3 L-Asparagine 132.0535 SMILES N[C@@H](CCC(O)=O)C(O)=O BiologicalState4 Adenosine diphosphate Glutamine synthetase 14265-44-2 CAS PYRUVATE BioCyc C6H9N3O2 L-Histidine 155.06947 58-64-0 CAS C02147 KEGG Compound Glutaminase liver isoform, mitochondrial L-serine dehydratase/L-threonine deaminase C4H7NO4 L-Aspartic acid 133.0375 C3H7NO3 L-Serine 105.042595 644102 PubChem-compound C6H6N2O2 Urocanic acid 138.04292 6083 PubChem-compound Histidine ammonia-lyase L-Aspartic acid SubPathwayInput CHEBI:18050 ChEBI H2O Water 18.010565 6-S-ACETYL-DIHYDROLIPOATE BioCyc SMILES NCC(O)=O Phosphoric acid Glycine SubPathwayOutput NADH BioCyc NAD BioCyc 1.0 56-84-8 CAS Glutamate dehydrogenase 1, mitochondrial Glutamate dehydrogenase 1, mitochondrial Aminomethyltransferase, mitochondrial 70-47-3 CAS SMILES [H][C@@]12CN(CN1C1=C(NC2)N=C(N)NC1=O)C1=CC=C(C=C1)C(=O)NC(CCC(O)=O)C(O)=O 30572 ChemSpider SMILES OC(=O)\C=C\C1=CNC=N1 Adenosine triphosphate ReactionCatalysis10 ACTIVATION C8H14O2S2 (R)-lipoic acid 206.04352 SMILES O H3N Ammonia 17.026548 C10H14N5O7P Adenosine monophosphate 347.06308 CHEBI:15971 ChEBI 4.3.1.3 false 4.3.1.3 L-Histidine ↔ Ammonia + Urocanic acid REVERSIBLE L-serine dehydratase/L-threonine deaminase 1004 PubChem-compound 5961 PubChem-compound 1.0 62-46-4 CAS Water 51 PubChem-compound CHEBI:15846 ChEBI P00367 UniProt ADP BioCyc C3H4O3 Pyruvic acid 88.016045 ReactionCatalysis38 ACTIVATION C16241 KEGG Compound ReactionCatalysis37 ACTIVATION 1.0 6038 ChemSpider GO:0005829 GENE ONTOLOGY C19H23N7O6 Tetrahydrofolic acid 445.171 274 ChemSpider P23378 UniProt 272 ChemSpider C5H6O5 Oxoglutaric acid 146.02153 HMDB00902 HMDB CHEBI:16908 ChEBI Ammonia O4P Phosphate 94.95342 Glutamine synthetase SER BioCyc ReactionCatalysis43 ACTIVATION 6031 ChemSpider Mitochondrion THF BioCyc GLY BioCyc 71-00-1 CAS 2.0 217 ChemSpider 1.0 58-68-4 CAS SMILES NC1=NC(=O)C2=C(NC[C@H](CNC3=CC=C(C=C3)C(=O)NC(CCC(O)=O)C(O)=O)N2)N1 SMILES [O-]P([O-])([O-])=O SMILES OC(=O)CCC(=O)C(O)=O 124-38-9 CAS CHEBI:30915 ChEBI C2H5NO2 Glycine 75.03203 L-Glutamine 5257127 PubChem-compound GO:0005759 GENE ONTOLOGY C20H23N7O6 5,10-Methylene-THF 457.171 GLN BioCyc 7664-41-7 CAS GLT BioCyc Isoaspartyl peptidase/L-asparaginase 462-20-4 CAS 1.8.1.4 false 1.8.1.4 Dihydrolipoate + NAD → (R)-lipoic acid + NADH LEFT_TO_RIGHT SMILES O=C=O 5951 PubChem-compound 222 PubChem-compound SMILES N[C@@H](CC(N)=O)C(O)=O 1.0 SMILES OP(O)(O)=O 2.0 O7P2 Pyrophosphate 173.91193 C21H29N7O14P2 NADH 665.12476 1.0 5957 PubChem-compound Reaction504 false L-Serine → Ammonia + Pyruvic acid LEFT_TO_RIGHT CHEBI:15636 ChEBI SubPathwayInteraction804 SubPathwayReaction SubPathway804Reaction SubPathwayInteraction805 SubPathway805Reaction SubPathwayReaction SMILES N[C@@H](CC1=CN=CN1)C(O)=O HMDB01096 HMDB SubPathwayInteraction802 SubPathway802Reaction SubPathwayReaction SubPathwayInteraction803 SubPathwayReaction SubPathway803Reaction SubPathwayInteraction808 SubPathwayReaction SubPathway808Reaction SubPathwayInteraction806 SubPathway806Reaction SubPathwayReaction SubPathwayInteraction807 SubPathwayReaction SubPathway807Reaction SMILES N[C@@H](CO)C(O)=O P48728 UniProt GO:0005739 GENE ONTOLOGY C10H15N5O10P2 Adenosine diphosphate 427.02942 GO:0005737 GENE ONTOLOGY SMILES N[C@@H](CC(O)=O)C(O)=O 1.0 ReactionCatalysis1160 ACTIVATION 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 1.0 5682 ChemSpider 5,10-Methylene-THF Mitochondrial Matrix 14000-31-8 CAS Glycine and Serine Metabolism SubPathway CARBAMOYL-P BioCyc Histidine Metabolism SubPathway Citric Acid Cycle SubPathway Urea Cycle SubPathway Glycine and Serine Metabolism SubPathway Glutamate Metabolism SubPathway Aspartate Metabolism SubPathway P08243 UniProt SMILES NC(=O)OP(O)(O)=O C8H16O2S2 Dihydrolipoate 208.05917 Cytoplasm Cell CHEBI:15422 ChEBI Glycine 1.0 HMDB00191 HMDB 388299 ChemSpider 127-17-3 CAS CHEBI:30817 ChEBI 1.0 1.4.4.2 false 1.4.4.2 (R)-lipoic acid + Glycine → 8-[(Aminomethyl)sulfanyl]-6-sulfanyloctanoic acid + Carbon dioxide LEFT_TO_RIGHT HIS BioCyc C5H9NO4 L-Glutamic acid 147.05316 33032 PubChem-compound 1.0 Dihydrolipoyl dehydrogenase, mitochondrial CHEBI:16526 ChEBI HMDB02142 HMDB CHEBI:16761 ChEBI P20132 UniProt CHEBI:15428 ChEBI 9606 TAXONOMY HMDB00177 HMDB 2-KETOGLUTARATE BioCyc HMDB00051 HMDB Oxoglutaric acid 5893 PubChem-compound HMDB02111 HMDB 1.0 1.0 P42357 UniProt SMP00067 SMPDB CHEBI:32816 ChEBI L-Serine SubPathwayOutput Ammonia Recycling 1.0 CHEBI:15377 ChEBI Glutaminase liver isoform, mitochondrial 278 PubChem-compound 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 56-45-1 CAS 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 Glycine dehydrogenase [decarboxylating], mitochondrial P31327 UniProt 1.0 HMDB00187 HMDB CHEBI:17672 ChEBI SMP00072 SMPDB Glutamine synthetase 280 PubChem-compound C9H19NO2S2 8-[(Aminomethyl)sulfanyl]-6-sulfanyloctanoic acid 237.08572 PW000009 PathWhiz Isoaspartyl peptidase/L-asparaginase L-Serine Dihydrolipoyl dehydrogenase, mitochondrial Carbamoyl phosphate 2.1.2.10 false 2.1.2.10 8-[(Aminomethyl)sulfanyl]-6-sulfanyloctanoic acid + Tetrahydrofolic acid → 5,10-Methylene-THF + Ammonia + Dihydrolipoate LEFT_TO_RIGHT ReactionCatalysis1498 ACTIVATION 736715 PubChem-compound HMDB01487 HMDB PW000002 PathWhiz ReactionCatalysis133 ACTIVATION ReactionCatalysis131 ACTIVATION ReactionCatalysis132 ACTIVATION PW000005 PathWhiz ReactionCatalysis130 ACTIVATION PW000003 PathWhiz SMILES N[C@@H](CCC(N)=O)C(O)=O L-serine dehydratase/L-threonine deaminase 1060 PubChem-compound HMDB00045 HMDB 1061 PubChem-compound HMDB00168 HMDB C00785 KEGG Compound 8-[(Aminomethyl)sulfanyl]-6-sulfanyloctanoic acid 1.0 13781857 PubChem-compound Dihydrolipoate ReactionCatalysis1157 ACTIVATION Glycine cleavage system H protein, mitochondrial L-serine dehydratase/L- threonine deaminase Isoaspartyl peptidase/L- asparaginase Asparagine synthetase [glutamine- hydrolyzing] Histidine ammonia-lyase Carbamoyl- phosphate synthase [ammonia], mitochondrial Glutamine synthetase Glutamate dehydrogenase 1, mitochondrial Glutaminase liver isoform, mitochondrial Aminomethyltransferase, mitochondrial Dihydrolipoyl dehydrogenase, mitochondrial Glycine dehydrogenase [decarboxylating], mitochondrial L-Serine L-Histidine Pyruvic acid Carbamoyl phosphate Glycine L-Glutamic acid L-Glutamic acid L-Aspartic acid NH 3 NH 3 L-Asparagine H 2 O ATP L-Glutamine H 2 O AMP PP i Urocanic acid ATP CO 2 H 2 O ADP P i ATP ADP P i L-Glutamine H 2 O NAD Oxoglutaric acid NADH H 2 O L-Glutamic acid Tetrahydrofolic acid 8-[(Aminomethyl)sulfanyl]-6- sulfanyloctanoic acid Dihydrolipoate 5,10-Methylene-THF NAD (R)-lipoic acid NADH CO 2 Pyridoxal 5'-phosphate Biotin Magnesium FAD Pyridoxal 5'-phosphate Glycine and Serine Metabolism Histidine Metabolism Citric Acid Cycle Urea Cycle Glycine and Serine Metabolism Glutamate Metabolism Glutamate Metabolism Aspartate Metabolism Glycine cleavage system Mitochondrial Matrix Extracellular Space Intracellular Space Mitochondria
CHEBI:16015 ChEBI 6022 PubChem-compound Q9UI32 UniProt 937 ChemSpider 1.0 HMDB00250 HMDB Aminomethyltransferase, mitochondrial CHEBI:16134 ChEBI L-Histidine 6267 PubChem-compound HMDB01341 HMDB CHEBI:30314 ChEBI 1.0 L-Aspartic acid 2.0 439153 PubChem-compound 50 ChemSpider CHEBI:20506 ChEBI 53481912 PubChem-compound CHEBI:17115 ChEBI CHEBI:16027 ChEBI HMDB00148 HMDB ATP BioCyc 18714427 ChemSpider 559142 ChemSpider Tetrahydrofolic acid Glycine dehydrogenase [decarboxylating], mitochondrial PW000043 PathWhiz 5746 ChemSpider P09622 UniProt 5745 ChemSpider ASN BioCyc 5742 ChemSpider L-ASPARTATE BioCyc 1.0 PW000162 PathWhiz 1.0 439175 PubChem-compound 1.0 SMP00044 SMPDB HMDB00243 HMDB 5858 ChemSpider L-Glutamic acid SubPathwayInput 5736 ChemSpider HMDB00123 HMDB 6274 PubChem-compound Adenosine monophosphate Carbon dioxide PW000157 PathWhiz METHYLENE-THF BioCyc C00101 KEGG Compound HMDB01451 HMDB HMDB12210 HMDB SMILES OC(=O)CCCCC(S)CCS Asparagine synthetase [glutamine-hydrolyzing] PPI BioCyc SMP00057 SMPDB SMP00059 SMPDB 643824 ChemSpider 1.0 5886 ChemSpider C5H10N2O3 L-Glutamine 146.06914 CH4NO5P Carbamoyl phosphate 140.98271 L-Glutamic acid Cytosol 7664-38-2 CAS 1.0 HMDB00208 HMDB Urocanic acid 6.0 1.0 C00009 KEGG Compound C00008 KEGG Compound C00001 KEGG Compound 1.0 HMDB13639 HMDB CHEBI:18361 ChEBI NADH CHEBI:18367 ChEBI C00004 KEGG Compound 1032 ChemSpider C00003 KEGG Compound 135-16-0 CAS 1031 ChemSpider C00002 KEGG Compound 388320 ChemSpider Carbamoyl-phosphate synthase [ammonia], mitochondrial HMDB01429 HMDB Reaction168 false L-Asparagine + Water ↔ Ammonia + L-Aspartic acid REVERSIBLE SMP00009 SMPDB Carbamoyl phosphate SubPathwayInput Pyruvic acid SubPathwayInput 1.0 1.0 C21H28N7O14P2 NAD 664.11694 44367445 PubChem-compound Adenosine triphosphate C00011 KEGG Compound L-Asparagine 2.0 C00014 KEGG Compound C00135 KEGG Compound C00013 KEGG Compound A8YXX4 UniProt Q7L266 UniProt 1.0 SMP00004 SMPDB Pyruvic acid 6112 PubChem-compound HMDB01533 HMDB Phosphate C00022 KEGG Compound C00143 KEGG Compound 56-65-5 CAS C00020 KEGG Compound 53-84-9 CAS C00026 KEGG Compound C00025 KEGG Compound CHEBI:26078 ChEBI SMILES NCSCCC(S)CCCCC(O)=O 3432-99-3 CAS 104-98-3 CAS L-Glutamic acid 6.3.1.2 false 6.3.1.2 Adenosine triphosphate + Ammonia + L-Glutamic acid → Adenosine diphosphate + L-Glutamine + Phosphate LEFT_TO_RIGHT 1.4.1.3 false 1.4.1.3 L-Glutamic acid + NAD + Water → Ammonia + NADH + Oxoglutaric acid LEFT_TO_RIGHT CL:0000000 CELL TYPE ONTOLOGY C00152 KEGG Compound 1.0 C00037 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 L-Histidine SubPathwayOutput AMP BioCyc Transport7 false Ammonia (→) Transport: Homo sapiens, Cell, Cytosol to Homo sapiens, Cell, Mitochondrial Matrix LEFT_TO_RIGHT SMILES [O-]P([O-])(=O)OP([O-])([O-])=O HMDB00301 HMDB 1.0 LIPOIC-ACID BioCyc CHEBI:17196 ChEBI 408 ChemSpider L-Glutamine C00049 KEGG Compound CHEBI:18047 ChEBI C00169 KEGG Compound 3.5.1.2 false 3.5.1.2 L-Glutamine + Water → Ammonia + L-Glutamic acid LEFT_TO_RIGHT 10.0 56-85-9 CAS Asparagine synthetase [glutamine-hydrolyzing] SMILES NC1=NC=NC2=C1N=CN2[C@@H]1O[C@H](COP(O)(=O)OP(O)(O)=O)[C@@H](O)[C@H]1O (R)-lipoic acid HMDB00538 HMDB 421 PubChem-compound Ammonia 7732-18-5 CAS 61-19-8 CAS SMILES NC1=C2N=CN([C@@H]3O[C@H](COP(O)(O)=O)[C@@H](O)[C@H]3O)C2=NC=N1 SMILES OC(=O)CCCC[C@@H]1CCSS1 1.0 AMMONIA BioCyc SMILES N 56-86-0 CAS 590-55-6 CAS Glutamate dehydrogenase 1, mitochondrial HMDB00641 HMDB 1.0 C10H16N5O13P3 Adenosine triphosphate 506.99576 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 56-40-6 CAS C00065 KEGG Compound C00064 KEGG Compound NAD Pyrophosphate SMILES CC(=O)C(O)=O 1.0 Ammonia HMDB01967 HMDB HMDB01846 HMDB UROCANATE BioCyc 962 PubChem-compound 5800 ChemSpider CO2 Carbon dioxide 43.98983 979 ChemSpider Carbamoyl-phosphate synthase [ammonia], mitochondrial 730 ChemSpider Water Glycine dehydrogenase [decarboxylating], mitochondrial Histidine ammonia-lyase H3O4P Phosphoric acid 97.9769 BiologicalState8 C4H8N2O3 L-Asparagine 132.0535 SMILES N[C@@H](CCC(O)=O)C(O)=O BiologicalState4 Adenosine diphosphate Glutamine synthetase 14265-44-2 CAS PYRUVATE BioCyc C6H9N3O2 L-Histidine 155.06947 58-64-0 CAS C02147 KEGG Compound Glutaminase liver isoform, mitochondrial L-serine dehydratase/L-threonine deaminase C4H7NO4 L-Aspartic acid 133.0375 C3H7NO3 L-Serine 105.042595 644102 PubChem-compound C6H6N2O2 Urocanic acid 138.04292 6083 PubChem-compound Histidine ammonia-lyase L-Aspartic acid SubPathwayInput CHEBI:18050 ChEBI H2O Water 18.010565 6-S-ACETYL-DIHYDROLIPOATE BioCyc SMILES NCC(O)=O Phosphoric acid Glycine SubPathwayOutput NADH BioCyc NAD BioCyc 1.0 56-84-8 CAS Glutamate dehydrogenase 1, mitochondrial Glutamate dehydrogenase 1, mitochondrial Aminomethyltransferase, mitochondrial 70-47-3 CAS SMILES [H][C@@]12CN(CN1C1=C(NC2)N=C(N)NC1=O)C1=CC=C(C=C1)C(=O)NC(CCC(O)=O)C(O)=O 30572 ChemSpider SMILES OC(=O)\C=C\C1=CNC=N1 Adenosine triphosphate ReactionCatalysis10 ACTIVATION C8H14O2S2 (R)-lipoic acid 206.04352 SMILES O H3N Ammonia 17.026548 C10H14N5O7P Adenosine monophosphate 347.06308 CHEBI:15971 ChEBI 4.3.1.3 false 4.3.1.3 L-Histidine ↔ Ammonia + Urocanic acid REVERSIBLE L-serine dehydratase/L-threonine deaminase 1004 PubChem-compound 5961 PubChem-compound 1.0 62-46-4 CAS Water 51 PubChem-compound CHEBI:15846 ChEBI P00367 UniProt ADP BioCyc C3H4O3 Pyruvic acid 88.016045 ReactionCatalysis38 ACTIVATION C16241 KEGG Compound ReactionCatalysis37 ACTIVATION 1.0 6038 ChemSpider GO:0005829 GENE ONTOLOGY C19H23N7O6 Tetrahydrofolic acid 445.171 274 ChemSpider P23378 UniProt 272 ChemSpider C5H6O5 Oxoglutaric acid 146.02153 HMDB00902 HMDB CHEBI:16908 ChEBI Ammonia O4P Phosphate 94.95342 Glutamine synthetase SER BioCyc ReactionCatalysis43 ACTIVATION 6031 ChemSpider Mitochondrion THF BioCyc GLY BioCyc 71-00-1 CAS 2.0 217 ChemSpider 1.0 58-68-4 CAS SMILES NC1=NC(=O)C2=C(NC[C@H](CNC3=CC=C(C=C3)C(=O)NC(CCC(O)=O)C(O)=O)N2)N1 SMILES [O-]P([O-])([O-])=O SMILES OC(=O)CCC(=O)C(O)=O 124-38-9 CAS CHEBI:30915 ChEBI C2H5NO2 Glycine 75.03203 L-Glutamine 5257127 PubChem-compound GO:0005759 GENE ONTOLOGY C20H23N7O6 5,10-Methylene-THF 457.171 GLN BioCyc 7664-41-7 CAS GLT BioCyc Isoaspartyl peptidase/L-asparaginase 462-20-4 CAS 1.8.1.4 false 1.8.1.4 Dihydrolipoate + NAD → (R)-lipoic acid + NADH LEFT_TO_RIGHT SMILES O=C=O 5951 PubChem-compound 222 PubChem-compound SMILES N[C@@H](CC(N)=O)C(O)=O 1.0 SMILES OP(O)(O)=O 2.0 O7P2 Pyrophosphate 173.91193 C21H29N7O14P2 NADH 665.12476 1.0 5957 PubChem-compound Reaction504 false L-Serine → Ammonia + Pyruvic acid LEFT_TO_RIGHT CHEBI:15636 ChEBI SubPathwayInteraction804 SubPathwayReaction SubPathway804Reaction SubPathwayInteraction805 SubPathway805Reaction SubPathwayReaction SMILES N[C@@H](CC1=CN=CN1)C(O)=O HMDB01096 HMDB SubPathwayInteraction802 SubPathway802Reaction SubPathwayReaction SubPathwayInteraction803 SubPathwayReaction SubPathway803Reaction SubPathwayInteraction808 SubPathwayReaction SubPathway808Reaction SubPathwayInteraction806 SubPathway806Reaction SubPathwayReaction SubPathwayInteraction807 SubPathwayReaction SubPathway807Reaction SMILES N[C@@H](CO)C(O)=O P48728 UniProt GO:0005739 GENE ONTOLOGY C10H15N5O10P2 Adenosine diphosphate 427.02942 GO:0005737 GENE ONTOLOGY SMILES N[C@@H](CC(O)=O)C(O)=O 1.0 ReactionCatalysis1160 ACTIVATION 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 1.0 5682 ChemSpider 5,10-Methylene-THF Mitochondrial Matrix 14000-31-8 CAS Glycine and Serine Metabolism SubPathway CARBAMOYL-P BioCyc Histidine Metabolism SubPathway Citric Acid Cycle SubPathway Urea Cycle SubPathway Glycine and Serine Metabolism SubPathway Glutamate Metabolism SubPathway Aspartate Metabolism SubPathway P08243 UniProt SMILES NC(=O)OP(O)(O)=O C8H16O2S2 Dihydrolipoate 208.05917 Cytoplasm Cell CHEBI:15422 ChEBI Glycine 1.0 HMDB00191 HMDB 388299 ChemSpider 127-17-3 CAS CHEBI:30817 ChEBI 1.0 1.4.4.2 false 1.4.4.2 (R)-lipoic acid + Glycine → 8-[(Aminomethyl)sulfanyl]-6-sulfanyloctanoic acid + Carbon dioxide LEFT_TO_RIGHT HIS BioCyc C5H9NO4 L-Glutamic acid 147.05316 33032 PubChem-compound 1.0 Dihydrolipoyl dehydrogenase, mitochondrial CHEBI:16526 ChEBI HMDB02142 HMDB CHEBI:16761 ChEBI P20132 UniProt CHEBI:15428 ChEBI 9606 TAXONOMY HMDB00177 HMDB 2-KETOGLUTARATE BioCyc HMDB00051 HMDB Oxoglutaric acid 5893 PubChem-compound HMDB02111 HMDB 1.0 1.0 P42357 UniProt SMP00067 SMPDB CHEBI:32816 ChEBI L-Serine SubPathwayOutput Ammonia Recycling 1.0 CHEBI:15377 ChEBI Glutaminase liver isoform, mitochondrial 278 PubChem-compound 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 56-45-1 CAS 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 Glycine dehydrogenase [decarboxylating], mitochondrial P31327 UniProt 1.0 HMDB00187 HMDB CHEBI:17672 ChEBI SMP00072 SMPDB Glutamine synthetase 280 PubChem-compound C9H19NO2S2 8-[(Aminomethyl)sulfanyl]-6-sulfanyloctanoic acid 237.08572 PW000009 PathWhiz Isoaspartyl peptidase/L-asparaginase L-Serine Dihydrolipoyl dehydrogenase, mitochondrial Carbamoyl phosphate 2.1.2.10 false 2.1.2.10 8-[(Aminomethyl)sulfanyl]-6-sulfanyloctanoic acid + Tetrahydrofolic acid → 5,10-Methylene-THF + Ammonia + Dihydrolipoate LEFT_TO_RIGHT ReactionCatalysis1498 ACTIVATION 736715 PubChem-compound HMDB01487 HMDB PW000002 PathWhiz ReactionCatalysis133 ACTIVATION ReactionCatalysis131 ACTIVATION ReactionCatalysis132 ACTIVATION PW000005 PathWhiz ReactionCatalysis130 ACTIVATION PW000003 PathWhiz SMILES N[C@@H](CCC(N)=O)C(O)=O L-serine dehydratase/L-threonine deaminase 1060 PubChem-compound HMDB00045 HMDB 1061 PubChem-compound HMDB00168 HMDB C00785 KEGG Compound 8-[(Aminomethyl)sulfanyl]-6-sulfanyloctanoic acid 1.0 13781857 PubChem-compound Dihydrolipoate ReactionCatalysis1157 ACTIVATION