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6022 PubChem-compound C23H38N7O17P3S Acetyl-CoA 809.1258 439161 PubChem-compound Mitochondrial pyruvate carrier 1 937 ChemSpider C00311 KEGG Compound Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial CHEBI:30797 ChEBI 439153 PubChem-compound 1.0 110-17-8 CAS HMDB0000094 HMDB 50 ChemSpider 1.0 PW000054 PathWhiz Succinyl-CoA ligase [GDP-forming] subunit beta, mitochondrial OXALACETIC_ACID BioCyc ATP BioCyc PW000051 PathWhiz 1.0 4.0 HMDB0001072 HMDB HMDB0001197 HMDB PW000046 PathWhiz PW000165 PathWhiz SMP0000547 SMPDB CHEBI:16238 ChEBI P09622 UniProt HCO3 BioCyc CHEBI:15380 ChEBI 5742 ChemSpider C25H40N7O19P3S Succinyl-CoA 867.1312 PW000163 PathWhiz Succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial PW000162 PathWhiz 1.0 1.0 5281915 PubChem-compound 97-67-6 CAS PW000155 PathWhiz C27H35N9O15P2 FADH 787.1728 Carbon dioxide 945 ChemSpider PW000152 PathWhiz PW000030 PathWhiz 1010 ChemSpider C10H15N5O11P2 Guanosine diphosphate 443.02432 1.0 SMILES CC(C)(COP(O)(=O)OP(O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1OP(O)(O)=O)N1C=NC2=C1N=CN=C2N)[C@@H](O)C(=O)NCCC(=O)NCCS CHEBI:30769 ChEBI Succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial CHEBI:30887 ChEBI 1.0 PW000029 PathWhiz SMILES CC(C)(COP(O)(=O)OP(O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1OP(O)(O)=O)N1C=NC2=C1N=CN=C2N)C(O)C(=O)NCCC(=O)NCCSC(=O)CCC(O)=O SubPathwayInteraction356 SubPathway356Reaction SubPathwayReaction SubPathwayInteraction357 SubPathwayReaction SubPathway357Reaction SubPathwayInteraction354 SubPathwayReaction SubPathway354Reaction SubPathwayInteraction355 SubPathway355Reaction SubPathwayReaction C10H16N5O14P3 Guanosine triphosphate 522.99066 SubPathwayInteraction358 SubPathwayReaction SubPathway358Reaction 1.0 SubPathwayInteraction359 SubPathway359Reaction SubPathwayReaction Probable 2-oxoglutarate dehydrogenase E1 component DHKTD1, mitochondrial 1161 ChemSpider Cytosol SUC BioCyc O43837 UniProt Reaction390 false L-Malic acid + NAD ↔ Hydrogen Ion + NADH + Oxalacetic acid REVERSIBLE 1.0 SubPathwayInteraction352 SubPathwayReaction SubPathway352Reaction SubPathwayInteraction353 SubPathway353Reaction SubPathwayReaction C00009 KEGG Compound C00008 KEGG Compound Succinate dehydrogenase C00001 KEGG Compound 1.0 559059 ChemSpider C00122 KEGG Compound SMILES CC1=CC2=C(C=C1C)N(C[C@H](O)[C@H](O)[C@H](O)COP(O)(=O)OP(O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1O)N1C=NC3=C1N=CN=C3N)C1=C(N2)C(=O)NC(=O)N1 4.0 NADH 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 Succinate dehydrogenase cytochrome b560 subunit, mitochondrial C00004 KEGG Compound CHEBI:18367 ChEBI C00003 KEGG Compound 1032 ChemSpider 1910-41-4 CAS 1031 ChemSpider C00002 KEGG Compound 60.0 Coenzyme A Mitochondrial Inner Membrane C11378 KEGG Compound SubPathwayInteraction378 SubPathway378Reaction SubPathwayReaction C59H90O4 Coenzyme Q10 862.6839 SubPathwayInteraction376 SubPathwayReaction SubPathway376Reaction SubPathwayInteraction377 SubPathwayReaction SubPathway377Reaction C27H33N9O15P2 FAD 785.1571 Pyruvic acid SubPathwayOutput SubPathwayInput C21H28N7O14P2 NAD 664.11694 1.0 C00011 KEGG Compound C00010 KEGG Compound HMDB0000902 HMDB Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial C00016 KEGG Compound FAD 1.0 SubPathwayInteraction370 SubPathway370Reaction SubPathwayReaction SubPathwayInteraction371 SubPathwayReaction SubPathway371Reaction SubPathwayInteraction374 SubPathway374Reaction SubPathwayReaction SubPathwayInteraction375 SubPathway375Reaction SubPathwayReaction FADH SubPathwayInput SubPathwayInteraction372 SubPathwayReaction SubPathway372Reaction SubPathwayInteraction373 SubPathway373Reaction SubPathwayReaction SubPathwayInteraction367 SubPathway367Reaction SubPathwayReaction H Hydrogen Ion 1.007825 Pyruvate dehydrogenase E1 component subunit beta, mitochondrial SubPathwayInteraction368 SubPathway368Reaction SubPathwayReaction SubPathwayInteraction365 SubPathwayReaction SubPathway365Reaction C00149 KEGG Compound SubPathwayInteraction366 SubPathwayReaction SubPathway366Reaction 388307 ChemSpider SubPathwayInteraction369 SubPathwayReaction SubPathway369Reaction Phosphate C00022 KEGG Compound C01352 KEGG Compound Pyruvate carboxylase, mitochondrial 56-65-5 CAS 53-84-9 CAS C00026 KEGG Compound C00024 KEGG Compound Fumaric acid SubPathwayOutput O14521 UniProt C00390 KEGG Compound SubPathwayInteraction360 SubPathway360Reaction SubPathwayReaction SubPathwayInteraction363 SubPathwayReaction SubPathway363Reaction SubPathwayInteraction364 SubPathway364Reaction SubPathwayReaction SubPathwayInteraction361 SubPathway361Reaction SubPathwayReaction SubPathwayInteraction362 SubPathway362Reaction SubPathwayReaction 1.0 769 PubChem-compound 56275-39-9 CAS Pyruvate carboxylase, mitochondrial C00158 KEGG Compound C00036 KEGG Compound Transport1 false Pyruvic acid (→) Transport: Homo sapiens, Cell, Cytosol to Homo sapiens, Cell, Mitochondrial Matrix LEFT_TO_RIGHT C00035 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 HMDB0000254 HMDB SMILES OC(=O)CCC(O)=O P50213 UniProt 1.0 SMILES OC(=O)C\C(=C\C(O)=O)C(O)=O HMDB0001341 HMDB P07954 UniProt 1.0 C00044 KEGG Compound C00042 KEGG Compound 4445197 ChemSpider 303-98-0 CAS C00288 KEGG Compound SMILES NC1=NC=NC2=C1N=CN2[C@@H]1O[C@H](COP(O)(=O)OP(O)(O)=O)[C@@H](O)[C@H]1O SMILES OC(=O)CC(O)(CC(O)=O)C(O)=O 604-98-8 CAS P51553 UniProt cis-Aconitic acid Succinyl-CoA 10197150 ChemSpider FUM BioCyc SMILES OC(=O)CC(=O)C(O)=O 7732-18-5 CAS Aconitate hydratase, mitochondrial CHEBI:18012 ChEBI HMDB0001487 HMDB HMDB0000156 HMDB Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex, mitochondrial Q99798 UniProt HMDB0001248 HMDB Pyruvic acid 1.0 Isocitrate dehydrogenase [NAD] subunit alpha, mitochondrial P21912 UniProt Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial 24.0 C10H16N5O13P3 Adenosine triphosphate 506.99576 SMILES COC1=C(O)C(C)=C(C\C=C(/C)CCC=C(C)C)C(O)=C1OC 305 ChemSpider NAD SMILES CC(=O)C(O)=O Isocitrate dehydrogenase [NAD] subunit gamma, mitochondrial 6.4.1.1 false 6.4.1.1 Adenosine triphosphate + Hydrogen carbonate + Pyruvic acid → Adenosine diphosphate + Oxalacetic acid + Phosphate LEFT_TO_RIGHT 962 PubChem-compound 1.0 5800 ChemSpider CO2 Carbon dioxide 43.98983 C00080 KEGG Compound Water Glutamate Metabolism SubPathway Ketone Body Metabolism SubPathway Q9Y5U8 UniProt HMDB0002111 HMDB HMDB0001022 HMDB Adenosine diphosphate 146-14-5 CAS 14265-44-2 CAS PYRUVATE BioCyc QH2 BioCyc 1.0 58-64-0 CAS GTP BioCyc 585-84-2 CAS C00091 KEGG Compound 1.0 P11177 UniProt HMDB0001273 HMDB Isocitric acid 444493 PubChem-compound Succinic acid SubPathwayInput Dihydrolipoyllysine-residue succinyltransferase component of 2-oxoglutarate dehydrogenase complex, mitochondrial ACETYL-COA BioCyc H2O Water 18.010565 1.0 FADH2 BioCyc Succinic acid HMDB0000193 HMDB Hydrogen carbonate HMDB0000072 HMDB NADH BioCyc NAD BioCyc HMDB0059597 HMDB Succinyl-CoA ligase [ADP/GDP-forming] subunit alpha, mitochondrial 749 ChemSpider 71-52-3 CAS 970 PubChem-compound CIS-ACONITATE BioCyc SMILES OC(=O)\C=C\C(O)=O Acetyl-CoA SubPathwayOutput SubPathwayInput Q99643 UniProt O75390 UniProt 146-91-8 CAS Citrate synthase, mitochondrial C4H6O5 L-Malic acid 134.02153 CHO3 Hydrogen carbonate 60.99257 446013 PubChem-compound C4H4O5 Oxalacetic acid 132.00587 L-Malic acid Succinyl-CoA ligase Adenosine triphosphate 1.0 Valine, Leucine, and Isoleucine Degradation SubPathway 2.0 Oxidation of Branched-Chain Fatty Acids SubPathway 21883788 PubChem-compound Citric acid SMILES O CIT BioCyc Guanosine triphosphate Lysine Degradation SubPathway Succinate dehydrogenase cytochrome b560 subunit, mitochondrial P11498 UniProt HMDB0000538 HMDB Succinyl-CoA ligase [GDP-forming] subunit beta, mitochondrial (C5H8)nC14H20O4 QH2 TransportCatalysis6 ACTIVATION Succinyl-CoA ligase [ADP/GDP-forming] subunit alpha, mitochondrial 1.0 328-42-7 CAS 6816 PubChem-compound 51 PubChem-compound Glycolysis SubPathway 2.0 SMILES O[C@@H](CC(O)=O)C(O)=O SMILES OC([O-])=O 1.0 CHEBI:15846 ChEBI ADP BioCyc C3H4O3 Pyruvic acid 88.016045 CHEBI:46245 ChEBI 1.0 C6H8O7 Isocitric acid 192.02701 1038 PubChem-compound Isocitrate dehydrogenase [NAD] subunit alpha, mitochondrial Oxoglutarate dehydrogenase complex Isocitrate dehydrogenase [NAD] subunit gamma, mitochondrial GO:0005829 GENE ONTOLOGY Alanine Metabolism SubPathway Isocitrate dehydrogenase [NAD] subunit beta, mitochondrial Fatty Acid Metabolism SubPathway 274 ChemSpider C5H6O5 Oxoglutaric acid 146.02153 393487 ChemSpider Citric acid Urea Cycle SubPathway CHEBI:16908 ChEBI Pyruvate dehydrogenase complex Pyruvate dehydrogenase E1 component subunit beta, mitochondrial O4P Phosphate 94.95342 6830 PubChem-compound 77-92-9 CAS 558863 ChemSpider Aspartate Metabolism SubPathway Tryptophan Metabolism SubPathway 1.0 Pyruvate Metabolism SubPathway Mitochondrion P40925 UniProt 643757 PubChem-compound Isocitrate dehydrogenase [NAD] subunit beta, mitochondrial HMDB0000208 HMDB 58-68-4 CAS SMP0000040 SMPDB HMDB0001423 HMDB Water SMILES [O-]P([O-])([O-])=O HMDB0001304 HMDB SMILES OC(=O)CCC(=O)C(O)=O Q96HY7 UniProt CHEBI:30915 ChEBI 124-38-9 CAS Guanosine diphosphate Mitochondrial Electron Transport Chain SubPathway ReactionCatalysis69 ACTIVATION 4.2.1.3 false 4.2.1.3 Citric acid ↔ Water + cis-Aconitic acid REVERSIBLE 1.0 ReactionCatalysis4 ACTIVATION HMDB0001429 HMDB ReactionCatalysis63 ACTIVATION ReactionCatalysis64 ACTIVATION ReactionCatalysis62 ACTIVATION ReactionCatalysis67 ACTIVATION ReactionCatalysis68 ACTIVATION SMP0000051 SMPDB HMDB0000223 HMDB GO:0005759 GENE ONTOLOGY GDP-4-DEHYDRO-6-DEOXY-D-MANNOSE BioCyc 4.2.1.3 false 4.2.1.3 Water + cis-Aconitic acid ↔ Isocitric acid REVERSIBLE Q96I99 UniProt 1.0 Malate-Aspartate Shuttle SubPathway SMP0000059 SMPDB 1.0 Gluconeogenesis SubPathway C4H4O4 Fumaric acid 116.010956 Tyrosine Metabolism SubPathway Glucose-Alanine Cycle SubPathway SMP0000055 SMPDB P31040 UniProt Aconitate hydratase, mitochondrial CHEBI:15741 ChEBI PW000523 PathWhiz Fumarate hydratase, mitochondrial Malate dehydrogenase, cytoplasmic SMILES O=C=O ReactionCatalysis75 ACTIVATION ReactionCatalysis78 ACTIVATION Mitochondrial pyruvate carrier 1 4444052 ChemSpider ReactionCatalysis79 ACTIVATION 1110 PubChem-compound Hydrogen Ion 1.0 HMDB0001201 HMDB HMDB0000595 HMDB SMP0000060 SMPDB C21H29N7O14P2 NADH 665.12476 ReactionCatalysis70 ACTIVATION 5280344 PubChem-compound HMDB0001206 HMDB 5957 PubChem-compound GO:0005743 GENE ONTOLOGY SMP0000067 SMPDB Propanoate Metabolism SubPathway C4H6O4 Succinic acid 118.02661 SMP0000063 SMPDB C6H6O6 cis-Aconitic acid 174.01643 320-77-4 CAS threo-d(s)-iso-citrate BioCyc Isocitric acid CHEBI:15996 ChEBI SMP0000072 SMPDB HMDB0000243 HMDB GO:0005739 GENE ONTOLOGY Arginine and Proline Metabolism SubPathway SMP0000071 SMPDB 60.0 SMILES OC(C(CC(O)=O)C(O)=O)C(O)=O C10H15N5O10P2 Adenosine diphosphate 427.02942 1.0 1.0 Malate dehydrogenase, cytoplasmic C6H8O7 Citric acid 192.02701 Probable 2-oxoglutarate dehydrogenase E1 component DHKTD1, mitochondrial SMP0000128 SMPDB SMP0000129 SMPDB SMP0000006 SMPDB SMP0000127 SMPDB SMILES CC1=CC2=C(C=C1C)N(C[C@H](O)[C@H](O)[C@H](O)CO[P@](O)(=O)O[P@@](O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1O)N1C=NC3=C1N=CN=C3N)C1=NC(=O)NC(=O)C1=N2 Dihydrolipoyllysine-residue succinyltransferase component of 2-oxoglutarate dehydrogenase complex, mitochondrial 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 19782904 PubChem-compound 1.0 SMILES COC1=C(OC)C(=O)C(C\C=C(/C)CC\C=C(/C)CC\C=C(/C)CC\C=C(/C)CC\C=C(/C)CC\C=C(/C)CC\C=C(/C)CC\C=C(/C)CC\C=C(/C)CCC=C(C)C)=C(C)C1=O 5682 ChemSpider CHEBI:1146900 ChEBI 1.2.4.2 false 1.2.4.2 Coenzyme A + NAD + Oxoglutaric acid → Carbon dioxide + Hydrogen Ion + NADH + Succinyl-CoA LEFT_TO_RIGHT 2.3.3.1 false 2.3.3.1 Acetyl-CoA + Oxalacetic acid + Water → Citric acid + Coenzyme A LEFT_TO_RIGHT Oxalacetic acid SubPathwayOutput SubPathwayInput 1.0 Mitochondrial Matrix 1.3.5.1 false 1.3.5.1 Coenzyme Q10 + FAD + Succinic acid ↔ FADH + Fumaric acid + QH2 REVERSIBLE Reaction593 false Guanosine diphosphate + Phosphate + Succinyl-CoA ↔ Coenzyme A + Guanosine triphosphate + Succinic acid REVERSIBLE 1.2.4.1 false 1.2.4.1 Coenzyme A + NAD + Pyruvic acid → Acetyl-CoA + Carbon dioxide + NADH LEFT_TO_RIGHT Fumarate hydratase, mitochondrial CO-A BioCyc CHEBI:17877 ChEBI SMP0000016 SMPDB 222656 PubChem-compound Fumaric acid 72-89-9 CAS 2.0 1078 ChemSpider 1.0 C21H36N7O16P3S Coenzyme A 767.11523 Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial CHEBI:15422 ChEBI Citrate synthase, mitochondrial 6557 ChemSpider 1.0 643975 PubChem-compound Homo sapiens SMILES CC(=O)SCCNC(=O)CCNC(=O)[C@H](O)C(C)(C)COP(O)(=O)OP(O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1OP(O)(O)=O)N1C=NC2=C1N=CN=C2N 388299 ChemSpider 127-17-3 CAS Coenzyme Q10 SMP0000020 SMPDB P36957 UniProt Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex, mitochondrial Dihydrolipoyl dehydrogenase, mitochondrial CHEBI:16526 ChEBI 8977 PubChem-compound 1.0 CHEBI:16761 ChEBI 4.2.1.2 false 4.2.1.2 L-Malic acid ↔ Fumaric acid + Water REVERSIBLE SMP0000037 SMPDB SMP0000032 SMPDB SMP0000030 SMPDB 9606 TAXONOMY PW000023 PathWhiz P08559 UniProt CHEBI:17544 ChEBI PW000142 PathWhiz PW000149 PathWhiz PW000028 PathWhiz 2-KETOGLUTARATE BioCyc 392413 ChemSpider 1.0 PW000146 PathWhiz Oxoglutaric acid SubPathwayOutput SubPathwayInput 8630 ChemSpider 5893 PubChem-compound P10515 UniProt Succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial 110-15-6 CAS Succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial Citrate synthase, mitochondrial CHEBI:32816 ChEBI 1.0 QH2 CHEBI:15377 ChEBI Fumarate hydratase, mitochondrial CHEBI:15378 ChEBI SMILES [H+] PW000010 PathWhiz Isocitrate dehydrogenase 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 60.0 CHEBI:17552 ChEBI Pyruvate carboxylase, mitochondrial 280 PubChem-compound CHEBI:30744 ChEBI Dihydrolipoyl dehydrogenase, mitochondrial CHEBI:32805 ChEBI PW000002 PathWhiz PW000001 PathWhiz 1.0 C00417 KEGG Compound PW000003 PathWhiz 1.0 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 1198 PubChem-compound 1.0 1.0 FAD BioCyc 193317 ChemSpider 3-METHYLBENZYLSUCCINYL-COA BioCyc 1.1.1.41 false 1.1.1.41 Isocitric acid + NAD → Carbon dioxide + Hydrogen Ion + NADH + Oxoglutaric acid LEFT_TO_RIGHT 86-01-1 CAS 1060 PubChem-compound 1061 PubChem-compound P53597 UniProt HMDB0001967 HMDB 1.0 CHEBI:15351 ChEBI SMP0000355 SMPDB Fumarase Deficiency 85-61-0 CAS UBIQUINONE-10 BioCyc Mitochondrial pyruvate carrier 1 Pyruvate dehydrogenase complex Citrate synthase, mitochondrial Isocitrate dehydrogenase [NAD] subunit alpha, mitochondrial Isocitrate dehydrogenase [NAD] subunit beta, mitochondrial Isocitrate dehydrogenase [NAD] subunit gamma, mitochondrial Oxoglutarate dehydrogenase complex Succinyl-CoA ligase Fumarate hydratase, mitochondrial Malate dehydrogenase, mitochondrial Pyruvate carboxylase, mitochondrial Succinate dehydrogenase Aconitate hydratase, mitochondrial Aconitate hydratase, mitochondrial Pyruvic acid Oxalacetic acid Accumulation Fumaric acid Succinic acid FADH 2 Accumulation Oxoglutaric acid Acetyl-CoA Pyruvic acid CoA NAD CO 2 NADH H 2 O Accumulation Citric acid CoA Isocitric acid NAD CO 2 NADH H + NAD CoA Succinyl-CoA NADH H + CO 2 P i GDP CoA GTP L-Malic acid H 2 O NAD NADH H + ATP HCO 3 - ADP P i Q FAD QH 2 cis-Aconitic acid H 2 O H 2 O Thiamine pyrophosphate Lipoamide FAD Magnesium Thiamine pyrophosphate Lipoamide FAD Biotin Manganese 4Fe-4S 4Fe-4S Gluconeogenesis Glycolysis Pyruvate Metabolism Malate- Aspartate Shuttle Gluconeogenesis Alanine Metabolism Aspartate Metabolism Pyruvate Metabolism Aspartate Metabolism Tyrosine Metabolism Arginine and Proline Metabolism Urea Cycle Propanoate Metabolism Mitochondrial Electron Transport Chain Malate- Aspartate Shuttle Glutamate Metabolism Alanine Metabolism Gluconeogenesis Glucose-Alanine Cycle Valine, Leucine, and Isoleucine Degradation Tryptophan Metabolism Pyruvate Metabolism Fatty Acid Metabolism Ketone Body Metabolism Lysine Degradation Oxidation of Branched-Chain Fatty Acids Propanoate Metabolism Inner mitochondrial membrane Mitochondrial intermembrane space Cytosol Mitochondrial matrix Mitochondrion Intracellular Space Inner mitochondrial membrane Outer mitochondrial membrane
6022 PubChem-compound C23H38N7O17P3S Acetyl-CoA 809.1258 439161 PubChem-compound Mitochondrial pyruvate carrier 1 937 ChemSpider C00311 KEGG Compound Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial CHEBI:30797 ChEBI 439153 PubChem-compound 1.0 110-17-8 CAS HMDB0000094 HMDB 50 ChemSpider 1.0 PW000054 PathWhiz Succinyl-CoA ligase [GDP-forming] subunit beta, mitochondrial OXALACETIC_ACID BioCyc ATP BioCyc PW000051 PathWhiz 1.0 4.0 HMDB0001072 HMDB HMDB0001197 HMDB PW000046 PathWhiz PW000165 PathWhiz SMP0000547 SMPDB CHEBI:16238 ChEBI P09622 UniProt HCO3 BioCyc CHEBI:15380 ChEBI 5742 ChemSpider C25H40N7O19P3S Succinyl-CoA 867.1312 PW000163 PathWhiz Succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial PW000162 PathWhiz 1.0 1.0 5281915 PubChem-compound 97-67-6 CAS PW000155 PathWhiz C27H35N9O15P2 FADH 787.1728 Carbon dioxide 945 ChemSpider PW000152 PathWhiz PW000030 PathWhiz 1010 ChemSpider C10H15N5O11P2 Guanosine diphosphate 443.02432 1.0 SMILES CC(C)(COP(O)(=O)OP(O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1OP(O)(O)=O)N1C=NC2=C1N=CN=C2N)[C@@H](O)C(=O)NCCC(=O)NCCS CHEBI:30769 ChEBI Succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial CHEBI:30887 ChEBI 1.0 PW000029 PathWhiz SMILES CC(C)(COP(O)(=O)OP(O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1OP(O)(O)=O)N1C=NC2=C1N=CN=C2N)C(O)C(=O)NCCC(=O)NCCSC(=O)CCC(O)=O SubPathwayInteraction356 SubPathway356Reaction SubPathwayReaction SubPathwayInteraction357 SubPathwayReaction SubPathway357Reaction SubPathwayInteraction354 SubPathwayReaction SubPathway354Reaction SubPathwayInteraction355 SubPathway355Reaction SubPathwayReaction C10H16N5O14P3 Guanosine triphosphate 522.99066 SubPathwayInteraction358 SubPathwayReaction SubPathway358Reaction 1.0 SubPathwayInteraction359 SubPathway359Reaction SubPathwayReaction Probable 2-oxoglutarate dehydrogenase E1 component DHKTD1, mitochondrial 1161 ChemSpider Cytosol SUC BioCyc O43837 UniProt Reaction390 false L-Malic acid + NAD ↔ Hydrogen Ion + NADH + Oxalacetic acid REVERSIBLE 1.0 SubPathwayInteraction352 SubPathwayReaction SubPathway352Reaction SubPathwayInteraction353 SubPathway353Reaction SubPathwayReaction C00009 KEGG Compound C00008 KEGG Compound Succinate dehydrogenase C00001 KEGG Compound 1.0 559059 ChemSpider C00122 KEGG Compound SMILES CC1=CC2=C(C=C1C)N(C[C@H](O)[C@H](O)[C@H](O)COP(O)(=O)OP(O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1O)N1C=NC3=C1N=CN=C3N)C1=C(N2)C(=O)NC(=O)N1 4.0 NADH 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 Succinate dehydrogenase cytochrome b560 subunit, mitochondrial C00004 KEGG Compound CHEBI:18367 ChEBI C00003 KEGG Compound 1032 ChemSpider 1910-41-4 CAS 1031 ChemSpider C00002 KEGG Compound 60.0 Coenzyme A Mitochondrial Inner Membrane C11378 KEGG Compound SubPathwayInteraction378 SubPathway378Reaction SubPathwayReaction C59H90O4 Coenzyme Q10 862.6839 SubPathwayInteraction376 SubPathwayReaction SubPathway376Reaction SubPathwayInteraction377 SubPathwayReaction SubPathway377Reaction C27H33N9O15P2 FAD 785.1571 Pyruvic acid SubPathwayOutput SubPathwayInput C21H28N7O14P2 NAD 664.11694 1.0 C00011 KEGG Compound C00010 KEGG Compound HMDB0000902 HMDB Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial C00016 KEGG Compound FAD 1.0 SubPathwayInteraction370 SubPathway370Reaction SubPathwayReaction SubPathwayInteraction371 SubPathwayReaction SubPathway371Reaction SubPathwayInteraction374 SubPathway374Reaction SubPathwayReaction SubPathwayInteraction375 SubPathway375Reaction SubPathwayReaction FADH SubPathwayInput SubPathwayInteraction372 SubPathwayReaction SubPathway372Reaction SubPathwayInteraction373 SubPathway373Reaction SubPathwayReaction SubPathwayInteraction367 SubPathway367Reaction SubPathwayReaction H Hydrogen Ion 1.007825 Pyruvate dehydrogenase E1 component subunit beta, mitochondrial SubPathwayInteraction368 SubPathway368Reaction SubPathwayReaction SubPathwayInteraction365 SubPathwayReaction SubPathway365Reaction C00149 KEGG Compound SubPathwayInteraction366 SubPathwayReaction SubPathway366Reaction 388307 ChemSpider SubPathwayInteraction369 SubPathwayReaction SubPathway369Reaction Phosphate C00022 KEGG Compound C01352 KEGG Compound Pyruvate carboxylase, mitochondrial 56-65-5 CAS 53-84-9 CAS C00026 KEGG Compound C00024 KEGG Compound Fumaric acid SubPathwayOutput O14521 UniProt C00390 KEGG Compound SubPathwayInteraction360 SubPathway360Reaction SubPathwayReaction SubPathwayInteraction363 SubPathwayReaction SubPathway363Reaction SubPathwayInteraction364 SubPathway364Reaction SubPathwayReaction SubPathwayInteraction361 SubPathway361Reaction SubPathwayReaction SubPathwayInteraction362 SubPathway362Reaction SubPathwayReaction 1.0 769 PubChem-compound 56275-39-9 CAS Pyruvate carboxylase, mitochondrial C00158 KEGG Compound C00036 KEGG Compound Transport1 false Pyruvic acid (→) Transport: Homo sapiens, Cell, Cytosol to Homo sapiens, Cell, Mitochondrial Matrix LEFT_TO_RIGHT C00035 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 HMDB0000254 HMDB SMILES OC(=O)CCC(O)=O P50213 UniProt 1.0 SMILES OC(=O)C\C(=C\C(O)=O)C(O)=O HMDB0001341 HMDB P07954 UniProt 1.0 C00044 KEGG Compound C00042 KEGG Compound 4445197 ChemSpider 303-98-0 CAS C00288 KEGG Compound SMILES NC1=NC=NC2=C1N=CN2[C@@H]1O[C@H](COP(O)(=O)OP(O)(O)=O)[C@@H](O)[C@H]1O SMILES OC(=O)CC(O)(CC(O)=O)C(O)=O 604-98-8 CAS P51553 UniProt cis-Aconitic acid Succinyl-CoA 10197150 ChemSpider FUM BioCyc SMILES OC(=O)CC(=O)C(O)=O 7732-18-5 CAS Aconitate hydratase, mitochondrial CHEBI:18012 ChEBI HMDB0001487 HMDB HMDB0000156 HMDB Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex, mitochondrial Q99798 UniProt HMDB0001248 HMDB Pyruvic acid 1.0 Isocitrate dehydrogenase [NAD] subunit alpha, mitochondrial P21912 UniProt Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial 24.0 C10H16N5O13P3 Adenosine triphosphate 506.99576 SMILES COC1=C(O)C(C)=C(C\C=C(/C)CCC=C(C)C)C(O)=C1OC 305 ChemSpider NAD SMILES CC(=O)C(O)=O Isocitrate dehydrogenase [NAD] subunit gamma, mitochondrial 6.4.1.1 false 6.4.1.1 Adenosine triphosphate + Hydrogen carbonate + Pyruvic acid → Adenosine diphosphate + Oxalacetic acid + Phosphate LEFT_TO_RIGHT 962 PubChem-compound 1.0 5800 ChemSpider CO2 Carbon dioxide 43.98983 C00080 KEGG Compound Water Glutamate Metabolism SubPathway Ketone Body Metabolism SubPathway Q9Y5U8 UniProt HMDB0002111 HMDB HMDB0001022 HMDB Adenosine diphosphate 146-14-5 CAS 14265-44-2 CAS PYRUVATE BioCyc QH2 BioCyc 1.0 58-64-0 CAS GTP BioCyc 585-84-2 CAS C00091 KEGG Compound 1.0 P11177 UniProt HMDB0001273 HMDB Isocitric acid 444493 PubChem-compound Succinic acid SubPathwayInput Dihydrolipoyllysine-residue succinyltransferase component of 2-oxoglutarate dehydrogenase complex, mitochondrial ACETYL-COA BioCyc H2O Water 18.010565 1.0 FADH2 BioCyc Succinic acid HMDB0000193 HMDB Hydrogen carbonate HMDB0000072 HMDB NADH BioCyc NAD BioCyc HMDB0059597 HMDB Succinyl-CoA ligase [ADP/GDP-forming] subunit alpha, mitochondrial 749 ChemSpider 71-52-3 CAS 970 PubChem-compound CIS-ACONITATE BioCyc SMILES OC(=O)\C=C\C(O)=O Acetyl-CoA SubPathwayOutput SubPathwayInput Q99643 UniProt O75390 UniProt 146-91-8 CAS Citrate synthase, mitochondrial C4H6O5 L-Malic acid 134.02153 CHO3 Hydrogen carbonate 60.99257 446013 PubChem-compound C4H4O5 Oxalacetic acid 132.00587 L-Malic acid Succinyl-CoA ligase Adenosine triphosphate 1.0 Valine, Leucine, and Isoleucine Degradation SubPathway 2.0 Oxidation of Branched-Chain Fatty Acids SubPathway 21883788 PubChem-compound Citric acid SMILES O CIT BioCyc Guanosine triphosphate Lysine Degradation SubPathway Succinate dehydrogenase cytochrome b560 subunit, mitochondrial P11498 UniProt HMDB0000538 HMDB Succinyl-CoA ligase [GDP-forming] subunit beta, mitochondrial (C5H8)nC14H20O4 QH2 TransportCatalysis6 ACTIVATION Succinyl-CoA ligase [ADP/GDP-forming] subunit alpha, mitochondrial 1.0 328-42-7 CAS 6816 PubChem-compound 51 PubChem-compound Glycolysis SubPathway 2.0 SMILES O[C@@H](CC(O)=O)C(O)=O SMILES OC([O-])=O 1.0 CHEBI:15846 ChEBI ADP BioCyc C3H4O3 Pyruvic acid 88.016045 CHEBI:46245 ChEBI 1.0 C6H8O7 Isocitric acid 192.02701 1038 PubChem-compound Isocitrate dehydrogenase [NAD] subunit alpha, mitochondrial Oxoglutarate dehydrogenase complex Isocitrate dehydrogenase [NAD] subunit gamma, mitochondrial GO:0005829 GENE ONTOLOGY Alanine Metabolism SubPathway Isocitrate dehydrogenase [NAD] subunit beta, mitochondrial Fatty Acid Metabolism SubPathway 274 ChemSpider C5H6O5 Oxoglutaric acid 146.02153 393487 ChemSpider Citric acid Urea Cycle SubPathway CHEBI:16908 ChEBI Pyruvate dehydrogenase complex Pyruvate dehydrogenase E1 component subunit beta, mitochondrial O4P Phosphate 94.95342 6830 PubChem-compound 77-92-9 CAS 558863 ChemSpider Aspartate Metabolism SubPathway Tryptophan Metabolism SubPathway 1.0 Pyruvate Metabolism SubPathway Mitochondrion P40925 UniProt 643757 PubChem-compound Isocitrate dehydrogenase [NAD] subunit beta, mitochondrial HMDB0000208 HMDB 58-68-4 CAS SMP0000040 SMPDB HMDB0001423 HMDB Water SMILES [O-]P([O-])([O-])=O HMDB0001304 HMDB SMILES OC(=O)CCC(=O)C(O)=O Q96HY7 UniProt CHEBI:30915 ChEBI 124-38-9 CAS Guanosine diphosphate Mitochondrial Electron Transport Chain SubPathway ReactionCatalysis69 ACTIVATION 4.2.1.3 false 4.2.1.3 Citric acid ↔ Water + cis-Aconitic acid REVERSIBLE 1.0 ReactionCatalysis4 ACTIVATION HMDB0001429 HMDB ReactionCatalysis63 ACTIVATION ReactionCatalysis64 ACTIVATION ReactionCatalysis62 ACTIVATION ReactionCatalysis67 ACTIVATION ReactionCatalysis68 ACTIVATION SMP0000051 SMPDB HMDB0000223 HMDB GO:0005759 GENE ONTOLOGY GDP-4-DEHYDRO-6-DEOXY-D-MANNOSE BioCyc 4.2.1.3 false 4.2.1.3 Water + cis-Aconitic acid ↔ Isocitric acid REVERSIBLE Q96I99 UniProt 1.0 Malate-Aspartate Shuttle SubPathway SMP0000059 SMPDB 1.0 Gluconeogenesis SubPathway C4H4O4 Fumaric acid 116.010956 Tyrosine Metabolism SubPathway Glucose-Alanine Cycle SubPathway SMP0000055 SMPDB P31040 UniProt Aconitate hydratase, mitochondrial CHEBI:15741 ChEBI PW000523 PathWhiz Fumarate hydratase, mitochondrial Malate dehydrogenase, cytoplasmic SMILES O=C=O ReactionCatalysis75 ACTIVATION ReactionCatalysis78 ACTIVATION Mitochondrial pyruvate carrier 1 4444052 ChemSpider ReactionCatalysis79 ACTIVATION 1110 PubChem-compound Hydrogen Ion 1.0 HMDB0001201 HMDB HMDB0000595 HMDB SMP0000060 SMPDB C21H29N7O14P2 NADH 665.12476 ReactionCatalysis70 ACTIVATION 5280344 PubChem-compound HMDB0001206 HMDB 5957 PubChem-compound GO:0005743 GENE ONTOLOGY SMP0000067 SMPDB Propanoate Metabolism SubPathway C4H6O4 Succinic acid 118.02661 SMP0000063 SMPDB C6H6O6 cis-Aconitic acid 174.01643 320-77-4 CAS threo-d(s)-iso-citrate BioCyc Isocitric acid CHEBI:15996 ChEBI SMP0000072 SMPDB HMDB0000243 HMDB GO:0005739 GENE ONTOLOGY Arginine and Proline Metabolism SubPathway SMP0000071 SMPDB 60.0 SMILES OC(C(CC(O)=O)C(O)=O)C(O)=O C10H15N5O10P2 Adenosine diphosphate 427.02942 1.0 1.0 Malate dehydrogenase, cytoplasmic C6H8O7 Citric acid 192.02701 Probable 2-oxoglutarate dehydrogenase E1 component DHKTD1, mitochondrial SMP0000128 SMPDB SMP0000129 SMPDB SMP0000006 SMPDB SMP0000127 SMPDB SMILES CC1=CC2=C(C=C1C)N(C[C@H](O)[C@H](O)[C@H](O)CO[P@](O)(=O)O[P@@](O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1O)N1C=NC3=C1N=CN=C3N)C1=NC(=O)NC(=O)C1=N2 Dihydrolipoyllysine-residue succinyltransferase component of 2-oxoglutarate dehydrogenase complex, mitochondrial 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 19782904 PubChem-compound 1.0 SMILES COC1=C(OC)C(=O)C(C\C=C(/C)CC\C=C(/C)CC\C=C(/C)CC\C=C(/C)CC\C=C(/C)CC\C=C(/C)CC\C=C(/C)CC\C=C(/C)CC\C=C(/C)CCC=C(C)C)=C(C)C1=O 5682 ChemSpider CHEBI:1146900 ChEBI 1.2.4.2 false 1.2.4.2 Coenzyme A + NAD + Oxoglutaric acid → Carbon dioxide + Hydrogen Ion + NADH + Succinyl-CoA LEFT_TO_RIGHT 2.3.3.1 false 2.3.3.1 Acetyl-CoA + Oxalacetic acid + Water → Citric acid + Coenzyme A LEFT_TO_RIGHT Oxalacetic acid SubPathwayOutput SubPathwayInput 1.0 Mitochondrial Matrix 1.3.5.1 false 1.3.5.1 Coenzyme Q10 + FAD + Succinic acid ↔ FADH + Fumaric acid + QH2 REVERSIBLE Reaction593 false Guanosine diphosphate + Phosphate + Succinyl-CoA ↔ Coenzyme A + Guanosine triphosphate + Succinic acid REVERSIBLE 1.2.4.1 false 1.2.4.1 Coenzyme A + NAD + Pyruvic acid → Acetyl-CoA + Carbon dioxide + NADH LEFT_TO_RIGHT Fumarate hydratase, mitochondrial CO-A BioCyc CHEBI:17877 ChEBI SMP0000016 SMPDB 222656 PubChem-compound Fumaric acid 72-89-9 CAS 2.0 1078 ChemSpider 1.0 C21H36N7O16P3S Coenzyme A 767.11523 Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial CHEBI:15422 ChEBI Citrate synthase, mitochondrial 6557 ChemSpider 1.0 643975 PubChem-compound Homo sapiens SMILES CC(=O)SCCNC(=O)CCNC(=O)[C@H](O)C(C)(C)COP(O)(=O)OP(O)(=O)OC[C@H]1O[C@H]([C@H](O)[C@@H]1OP(O)(O)=O)N1C=NC2=C1N=CN=C2N 388299 ChemSpider 127-17-3 CAS Coenzyme Q10 SMP0000020 SMPDB P36957 UniProt Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex, mitochondrial Dihydrolipoyl dehydrogenase, mitochondrial CHEBI:16526 ChEBI 8977 PubChem-compound 1.0 CHEBI:16761 ChEBI 4.2.1.2 false 4.2.1.2 L-Malic acid ↔ Fumaric acid + Water REVERSIBLE SMP0000037 SMPDB SMP0000032 SMPDB SMP0000030 SMPDB 9606 TAXONOMY PW000023 PathWhiz P08559 UniProt CHEBI:17544 ChEBI PW000142 PathWhiz PW000149 PathWhiz PW000028 PathWhiz 2-KETOGLUTARATE BioCyc 392413 ChemSpider 1.0 PW000146 PathWhiz Oxoglutaric acid SubPathwayOutput SubPathwayInput 8630 ChemSpider 5893 PubChem-compound P10515 UniProt Succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial 110-15-6 CAS Succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial Citrate synthase, mitochondrial CHEBI:32816 ChEBI 1.0 QH2 CHEBI:15377 ChEBI Fumarate hydratase, mitochondrial CHEBI:15378 ChEBI SMILES [H+] PW000010 PathWhiz Isocitrate dehydrogenase 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 60.0 CHEBI:17552 ChEBI Pyruvate carboxylase, mitochondrial 280 PubChem-compound CHEBI:30744 ChEBI Dihydrolipoyl dehydrogenase, mitochondrial CHEBI:32805 ChEBI PW000002 PathWhiz PW000001 PathWhiz 1.0 C00417 KEGG Compound PW000003 PathWhiz 1.0 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 1198 PubChem-compound 1.0 1.0 FAD BioCyc 193317 ChemSpider 3-METHYLBENZYLSUCCINYL-COA BioCyc 1.1.1.41 false 1.1.1.41 Isocitric acid + NAD → Carbon dioxide + Hydrogen Ion + NADH + Oxoglutaric acid LEFT_TO_RIGHT 86-01-1 CAS 1060 PubChem-compound 1061 PubChem-compound P53597 UniProt HMDB0001967 HMDB 1.0 CHEBI:15351 ChEBI SMP0000355 SMPDB