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HMDB00254 HMDB HMDB00134 HMDB 6022 PubChem-compound C23H38N7O17P3S Acetyl-CoA 809.1258 439161 PubChem-compound Mitochondrial pyruvate carrier 1 937 ChemSpider C00311 KEGG Compound HMDB01341 HMDB Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial CHEBI:30797 ChEBI 439153 PubChem-compound 1.0 110-17-8 CAS 50 ChemSpider SMP00020 SMPDB 1.0 PW000054 PathWhiz Succinyl-CoA ligase [GDP-forming] subunit beta, mitochondrial OXALACETIC_ACID BioCyc ATP BioCyc PW000051 PathWhiz 1.0 4.0 SMP00037 SMPDB SMP00030 SMPDB SMP00032 SMPDB HMDB01201 HMDB HMDB00595 HMDB PW000046 PathWhiz PW000165 PathWhiz CHEBI:16238 ChEBI P09622 UniProt HMDB01206 HMDB 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 SMP00040 SMPDB HMDB59597 HMDB 1.0 5281915 PubChem-compound 97-67-6 CAS HMDB00243 HMDB 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(O)C(=O)NCCC(=O)NCCS SMP00051 SMPDB CHEBI:30769 ChEBI Succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial CHEBI:30887 ChEBI 1.0 SMP00059 SMPDB PW000029 PathWhiz SMP00055 SMPDB 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 HMDB01423 HMDB SubPathwayInteraction354 SubPathwayReaction SubPathway354Reaction SubPathwayInteraction355 SubPathway355Reaction SubPathwayReaction C10H16N5O14P3 Guanosine triphosphate 522.99066 HMDB01304 HMDB 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 HMDB00208 HMDB SubPathwayInteraction352 SubPathwayReaction SubPathway352Reaction SubPathwayInteraction353 SubPathway353Reaction SubPathwayReaction C00009 KEGG Compound C00008 KEGG Compound HMDB00223 HMDB 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 HMDB01429 HMDB SMP00355 SMPDB 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 SMP00006 SMPDB SMP00127 SMPDB SMP00128 SMPDB SMP00129 SMPDB C00011 KEGG Compound C00010 KEGG Compound 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 SMP00016 SMPDB 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 SMP00546 SMPDB CL:0000000 CELL TYPE ONTOLOGY 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 SMILES OC(=O)CCC(O)=O P50213 UniProt Gluconeogenesis SubPathway 1.0 Glycolysis SubPathway Pyruvate Metabolism SubPathway Malate-Aspartate Shuttle SubPathway SMILES OC(=O)C\C(=C\C(O)=O)C(O)=O Gluconeogenesis SubPathway Alanine Metabolism SubPathway Aspartate Metabolism SubPathway Pyruvate Metabolism SubPathway 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 Aspartate Metabolism SubPathway Tyrosine Metabolism SubPathway Arginine and Proline Metabolism SubPathway Urea Cycle SubPathway HMDB00538 HMDB Propanoate Metabolism SubPathway P51553 UniProt cis-Aconitic acid Mitochondrial Electron Transport Chain SubPathway Malate-Aspartate Shuttle SubPathway Glutamate Metabolism SubPathway Alanine Metabolism SubPathway Gluconeogenesis SubPathway Succinyl-CoA 10197150 ChemSpider FUM BioCyc SMILES OC(=O)CC(=O)C(O)=O 7732-18-5 CAS Aconitate hydratase, mitochondrial CHEBI:18012 ChEBI Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex, mitochondrial Q99798 UniProt Glucose-Alanine Cycle SubPathway Valine, Leucine and Isoleucine Degradation SubPathway Pyruvic acid Tryptophan Metabolism SubPathway Pyruvate Metabolism SubPathway 1.0 Isocitrate dehydrogenase [NAD] subunit alpha, mitochondrial Fatty acid Metabolism SubPathway Ketone Body Metabolism SubPathway Lysine Degradation SubPathway Oxidation of Branched Chain Fatty Acids SubPathway P21912 UniProt Propanoate Metabolism SubPathway Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial BiologicalState17 24.0 C10H16N5O13P3 Adenosine triphosphate 506.99576 SMILES COC1=C(O)C(C)=C(CC=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 HMDB01967 HMDB 962 PubChem-compound 1.0 5800 ChemSpider CO2 Carbon dioxide 43.98983 C00080 KEGG Compound Water Q9Y5U8 UniProt BiologicalState3 BiologicalState4 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 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 Hydrogen carbonate NADH BioCyc NAD BioCyc 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 2.0 21883788 PubChem-compound Citric acid SMILES O CIT BioCyc Guanosine triphosphate Succinate dehydrogenase cytochrome b560 subunit, mitochondrial P11498 UniProt Succinyl-CoA ligase [GDP-forming] subunit beta, mitochondrial C19H28O4 QH2 320.19876 TransportCatalysis6 ACTIVATION Succinyl-CoA ligase [ADP/GDP-forming] subunit alpha, mitochondrial 1.0 328-42-7 CAS 6816 PubChem-compound 51 PubChem-compound 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 Isocitrate dehydrogenase [NAD] subunit beta, mitochondrial 274 ChemSpider C5H6O5 Oxoglutaric acid 146.02153 HMDB00902 HMDB 393487 ChemSpider Citric acid 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 1.0 Mitochondrion P40925 UniProt 643757 PubChem-compound Isocitrate dehydrogenase [NAD] subunit beta, mitochondrial 58-68-4 CAS Water SMILES [O-]P([O-])([O-])=O SMILES OC(=O)CCC(=O)C(O)=O Q96HY7 UniProt CHEBI:30915 ChEBI 124-38-9 CAS Guanosine diphosphate Congenital lactic acidosis ReactionCatalysis69 ACTIVATION 4.2.1.3 false 4.2.1.3 Citric acid ↔ Water + cis-Aconitic acid REVERSIBLE 1.0 ReactionCatalysis4 ACTIVATION ReactionCatalysis63 ACTIVATION ReactionCatalysis64 ACTIVATION ReactionCatalysis62 ACTIVATION ReactionCatalysis67 ACTIVATION ReactionCatalysis68 ACTIVATION 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 1.0 C4H4O4 Fumaric acid 116.010956 P31040 UniProt Aconitate hydratase, mitochondrial CHEBI:15741 ChEBI Fumarate hydratase, mitochondrial PW000522 PathWhiz 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 C21H29N7O14P2 NADH 665.12476 ReactionCatalysis70 ACTIVATION 5280344 PubChem-compound 5957 PubChem-compound GO:0005743 GENE ONTOLOGY C4H6O4 Succinic acid 118.02661 C6H6O6 cis-Aconitic acid 174.01643 320-77-4 CAS threo-d(s)-iso-citrate BioCyc Isocitric acid CHEBI:15996 ChEBI GO:0005739 GENE ONTOLOGY 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 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 HMDB00094 HMDB 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 HMDB01072 HMDB 222656 PubChem-compound Fumaric acid 72-89-9 CAS 2.0 1078 ChemSpider 1.0 HMDB01197 HMDB C21H36N7O16P3S Coenzyme A 767.11523 Cell Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial CHEBI:15422 ChEBI Citrate synthase, mitochondrial 6557 ChemSpider 1.0 643975 PubChem-compound HMDB00193 HMDB HMDB00072 HMDB 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 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 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 HMDB02111 HMDB HMDB01022 HMDB P10515 UniProt Succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial SMP00060 SMPDB 110-15-6 CAS Succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial Citrate synthase, mitochondrial SMP00067 SMPDB CHEBI:32816 ChEBI SMP00063 SMPDB 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 HMDB01273 HMDB Pyruvate carboxylase, mitochondrial SMP00071 SMPDB SMP00072 SMPDB 280 PubChem-compound CHEBI:30744 ChEBI Dihydrolipoyl dehydrogenase, mitochondrial CHEBI:32805 ChEBI HMDB01487 HMDB PW000002 PathWhiz HMDB00156 HMDB PW000001 PathWhiz 1.0 HMDB01248 HMDB 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 1.0 CHEBI:15351 ChEBI 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 Accumulation Pyruvic acid Oxalacetic acid Fumaric acid Succinic acid FADH 2 Oxoglutaric acid Acetyl-CoA Accumulation Pyruvic acid CoA NAD CO 2 NADH H 2 O 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
HMDB00254 HMDB HMDB00134 HMDB 6022 PubChem-compound C23H38N7O17P3S Acetyl-CoA 809.1258 439161 PubChem-compound Mitochondrial pyruvate carrier 1 937 ChemSpider C00311 KEGG Compound HMDB01341 HMDB Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial CHEBI:30797 ChEBI 439153 PubChem-compound 1.0 110-17-8 CAS 50 ChemSpider http://identifiers.org/smpdb/SMP00020 SMPDB 1.0 PW000054 PathWhiz Succinyl-CoA ligase [GDP-forming] subunit beta, mitochondrial OXALACETIC_ACID BioCyc ATP BioCyc PW000051 PathWhiz 1.0 4.0 http://identifiers.org/smpdb/SMP00037 SMPDB http://identifiers.org/smpdb/SMP00030 SMPDB http://identifiers.org/smpdb/SMP00032 SMPDB HMDB01201 HMDB HMDB00595 HMDB PW000046 PathWhiz PW000165 PathWhiz CHEBI:16238 ChEBI P09622 UniProt HMDB01206 HMDB 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 http://identifiers.org/smpdb/SMP00040 SMPDB HMDB59597 HMDB 1.0 5281915 PubChem-compound 97-67-6 CAS HMDB00243 HMDB 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(O)C(=O)NCCC(=O)NCCS http://identifiers.org/smpdb/SMP00051 SMPDB CHEBI:30769 ChEBI Succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial CHEBI:30887 ChEBI 1.0 http://identifiers.org/smpdb/SMP00059 SMPDB PW000029 PathWhiz http://identifiers.org/smpdb/SMP00055 SMPDB 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 HMDB01423 HMDB SubPathwayInteraction354 SubPathwayReaction SubPathway354Reaction SubPathwayInteraction355 SubPathway355Reaction SubPathwayReaction C10H16N5O14P3 Guanosine triphosphate 522.99066 HMDB01304 HMDB 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 HMDB00208 HMDB SubPathwayInteraction352 SubPathwayReaction SubPathway352Reaction SubPathwayInteraction353 SubPathway353Reaction SubPathwayReaction C00009 KEGG Compound C00008 KEGG Compound HMDB00223 HMDB 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 HMDB01429 HMDB http://identifiers.org/smpdb/SMP00355 SMPDB 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 http://identifiers.org/smpdb/SMP00006 SMPDB http://identifiers.org/smpdb/SMP00127 SMPDB http://identifiers.org/smpdb/SMP00128 SMPDB http://identifiers.org/smpdb/SMP00129 SMPDB C00011 KEGG Compound C00010 KEGG Compound 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 http://identifiers.org/smpdb/SMP00016 SMPDB 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 http://identifiers.org/smpdb/SMP00546 SMPDB CL:0000000 CELL TYPE ONTOLOGY 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 SMILES OC(=O)CCC(O)=O P50213 UniProt Gluconeogenesis SubPathway 1.0 Glycolysis SubPathway Pyruvate Metabolism SubPathway Malate-Aspartate Shuttle SubPathway SMILES OC(=O)C\C(=C\C(O)=O)C(O)=O Gluconeogenesis SubPathway Alanine Metabolism SubPathway Aspartate Metabolism SubPathway Pyruvate Metabolism SubPathway 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 Aspartate Metabolism SubPathway Tyrosine Metabolism SubPathway Arginine and Proline Metabolism SubPathway Urea Cycle SubPathway HMDB00538 HMDB Propanoate Metabolism SubPathway P51553 UniProt cis-Aconitic acid Mitochondrial Electron Transport Chain SubPathway Malate-Aspartate Shuttle SubPathway Glutamate Metabolism SubPathway Alanine Metabolism SubPathway Gluconeogenesis SubPathway Succinyl-CoA 10197150 ChemSpider FUM BioCyc SMILES OC(=O)CC(=O)C(O)=O 7732-18-5 CAS Aconitate hydratase, mitochondrial CHEBI:18012 ChEBI Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex, mitochondrial Q99798 UniProt Glucose-Alanine Cycle SubPathway Valine, Leucine and Isoleucine Degradation SubPathway Pyruvic acid Tryptophan Metabolism SubPathway Pyruvate Metabolism SubPathway 1.0 Isocitrate dehydrogenase [NAD] subunit alpha, mitochondrial Fatty acid Metabolism SubPathway Ketone Body Metabolism SubPathway Lysine Degradation SubPathway Oxidation of Branched Chain Fatty Acids SubPathway P21912 UniProt Propanoate Metabolism SubPathway Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial BiologicalState17 Homo sapiens, Cell, Mitochondrial Inner Membrane 24.0 C10H16N5O13P3 Adenosine triphosphate 506.99576 SMILES COC1=C(O)C(C)=C(CC=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 HMDB01967 HMDB 962 PubChem-compound 1.0 5800 ChemSpider CO2 Carbon dioxide 43.98983 C00080 KEGG Compound Water Q9Y5U8 UniProt BiologicalState3 Homo sapiens, Cell, Mitochondrion BiologicalState4 Homo sapiens, Cell, Mitochondrial Matrix 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 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 Hydrogen carbonate NADH BioCyc NAD BioCyc 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 2.0 21883788 PubChem-compound Citric acid SMILES O CIT BioCyc Guanosine triphosphate Succinate dehydrogenase cytochrome b560 subunit, mitochondrial P11498 UniProt Succinyl-CoA ligase [GDP-forming] subunit beta, mitochondrial C19H28O4 QH2 320.19876 TransportCatalysis6 ACTIVATION Succinyl-CoA ligase [ADP/GDP-forming] subunit alpha, mitochondrial 1.0 328-42-7 CAS 6816 PubChem-compound 51 PubChem-compound 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 Isocitrate dehydrogenase [NAD] subunit beta, mitochondrial 274 ChemSpider C5H6O5 Oxoglutaric acid 146.02153 HMDB00902 HMDB 393487 ChemSpider Citric acid 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 1.0 Mitochondrion P40925 UniProt 643757 PubChem-compound Isocitrate dehydrogenase [NAD] subunit beta, mitochondrial 58-68-4 CAS Water SMILES [O-]P([O-])([O-])=O SMILES OC(=O)CCC(=O)C(O)=O Q96HY7 UniProt CHEBI:30915 ChEBI 124-38-9 CAS Guanosine diphosphate Congenital lactic acidosis ReactionCatalysis69 ACTIVATION 4.2.1.3 false 4.2.1.3 Citric acid ↔ Water + cis-Aconitic acid REVERSIBLE 1.0 ReactionCatalysis4 ACTIVATION ReactionCatalysis63 ACTIVATION ReactionCatalysis64 ACTIVATION ReactionCatalysis62 ACTIVATION ReactionCatalysis67 ACTIVATION ReactionCatalysis68 ACTIVATION 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 1.0 C4H4O4 Fumaric acid 116.010956 P31040 UniProt Aconitate hydratase, mitochondrial CHEBI:15741 ChEBI Fumarate hydratase, mitochondrial PW000522 PathWhiz 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 C21H29N7O14P2 NADH 665.12476 ReactionCatalysis70 ACTIVATION 5280344 PubChem-compound 5957 PubChem-compound GO:0005743 GENE ONTOLOGY C4H6O4 Succinic acid 118.02661 C6H6O6 cis-Aconitic acid 174.01643 320-77-4 CAS threo-d(s)-iso-citrate BioCyc Isocitric acid CHEBI:15996 ChEBI GO:0005739 GENE ONTOLOGY 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 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 HMDB00094 HMDB 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 HMDB01072 HMDB 222656 PubChem-compound Fumaric acid