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5605 ChemSpider 1.0 6022 PubChem-compound C23H38N7O17P3S Acetyl-CoA 809.1258 PW002360 PathWhiz 439161 PubChem-compound Mitochondrial pyruvate carrier 1 937 ChemSpider C00311 KEGG Compound Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial CHEBI:30797 ChEBI 1.0 439153 PubChem-compound Mitochondrial dicarboxylate carrier 110-17-8 CAS Q96KS0 UniProt HMDB0000094 HMDB 50 ChemSpider 1.0 Succinyl-CoA ligase [GDP-forming] subunit beta, mitochondrial OXALACETIC_ACID BioCyc ATP BioCyc 1.0 Coenzyme Q10 FAD 4.0 HMDB0001072 HMDB 42 ChemSpider PRO BioCyc 1.0 HMDB0001197 HMDB Isocitrate dehydrogenase [NADP], mitochondrial mutant Isocitrate dehydrogenase 2 [NADP], mitochondrial PW000165 PathWhiz CHEBI:17203 ChEBI CHEBI:16238 ChEBI P09622 UniProt NADP+-specific isocitrate dehydrogenase HCO3 BioCyc CHEBI:15380 ChEBI 5742 ChemSpider C25H40N7O19P3S Succinyl-CoA 867.1312 1.0 CHEBI:16474 ChEBI C02630 KEGG Compound 952 ChemSpider 1.0 Carbon dioxide 1.0 1.0 5281915 PubChem-compound 97-67-6 CAS 2-Hydroxyglutarate C27H35N9O15P2 FADH 787.1728 Carbon dioxide 945 ChemSpider 1010 ChemSpider C10H15N5O11P2 Guanosine diphosphate 443.02432 O75874 UniProt 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 1.0 CHEBI:30769 ChEBI Succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial CHEBI:30887 ChEBI 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)NCCSC(=O)CCC(O)=O 1.0 C10H16N5O14P3 Guanosine triphosphate 522.99066 1161 ChemSpider Probable 2-oxoglutarate dehydrogenase E1 component DHKTD1, mitochondrial Cytosol SUC BioCyc O43837 UniProt Reaction390 false L-Malic acid + NAD ↔ Hydrogen Ion + NADH + Oxalacetic acid REVERSIBLE 1.0 C00009 KEGG Compound C00008 KEGG Compound C00007 KEGG Compound C00006 KEGG Compound CHEBI:18009 ChEBI 1.0 C00001 KEGG Compound C00122 KEGG Compound 559059 ChemSpider 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 C00005 KEGG Compound 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 C59H90O4 Coenzyme Q10 862.6839 Pyruvic acid SubPathwayOutput C27H33N9O15P2 FAD 785.1571 C21H28N7O14P2 NAD 664.11694 1.0 C00011 KEGG Compound C00010 KEGG Compound 1.0 Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial HMDB0000902 HMDB C00016 KEGG Compound 1.0 1.0 NAD(P) BioCyc FADH SubPathwayInput Pyruvate dehydrogenase E1 component subunit beta, mitochondrial H Hydrogen Ion 1.007825 C00149 KEGG Compound 388307 ChemSpider Phosphate C00022 KEGG Compound Pyruvate carboxylase, mitochondrial 56-65-5 CAS C01352 KEGG Compound 53-84-9 CAS C00148 KEGG Compound C00026 KEGG Compound C00024 KEGG Compound mutant succinate dehydrogenase Fumaric acid O14521 UniProt C00390 KEGG Compound 1.0 1.0 769 PubChem-compound The Oncogenic Action of Succinate Pyruvate carboxylase, mitochondrial 56275-39-9 CAS 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 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 328-50-7 CAS HMDB0000134 HMDB HMDB0000254 HMDB SMILES OC(=O)CCC(O)=O Transport517 false Succinic acid (→) Transport: Homo sapiens, Cell, Mitochondrial Inner Membrane to Homo sapiens, Cytosol LEFT_TO_RIGHT 1.0 P50213 UniProt 1.0 SMILES OC(=O)C\C(=C\C(O)=O)C(O)=O HMDB0001341 HMDB prolyl hydroxylase 4-Hydroxyproline P07954 UniProt 1.0 C00044 KEGG Compound 128566 ChemSpider C00042 KEGG Compound 4445197 ChemSpider C00288 KEGG Compound 303-98-0 CAS SMILES NC1=NC=NC2=C1N=CN2[C@@H]1O[C@H](COP(O)(=O)OP(O)(O)=O)[C@@H](O)[C@H]1O SMILES OC(=O)CC(O)(CC(O)=O)C(O)=O 604-98-8 CAS P51553 UniProt cis-Aconitic acid Succinyl-CoA 2889-31-8 CAS 10197150 ChemSpider FUM BioCyc SMILES OC(=O)CC(=O)C(O)=O 7732-18-5 CAS Aconitate hydratase, mitochondrial CHEBI:18012 ChEBI Cytoplasmic aconitate hydratase 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 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 SubPathwayInteraction3377 SubPathway3377Reaction SubPathwayReaction NAD 305 ChemSpider C01157 KEGG Compound HMDB0001377 HMDB SMILES CC(=O)C(O)=O Isocitrate dehydrogenase [NAD] subunit gamma, mitochondrial 1.0 Mitochondrial pyruvate carrier 1 6.4.1.1 false 6.4.1.1 Adenosine triphosphate + Hydrogen carbonate + Pyruvic acid → Adenosine diphosphate + Oxalacetic acid + Phosphate LEFT_TO_RIGHT HMDB0000162 HMDB 962 PubChem-compound 1.0 C5H9NO2 L-Proline 115.06333 5800 ChemSpider H0YLL5 UniProt CO2 Carbon dioxide 43.98983 145742 PubChem-compound 1.1.1.42 false 1.1.1.42 Isocitric acid + NADP → NADPH + Oxoglutaric acid LEFT_TO_RIGHT C00080 KEGG Compound 1.3.5.1 false 1.3.5.1 Coenzyme Q10 + FAD + Succinic acid ↔ FADH + Fumaric acid + QH2 REVERSIBLE SMILES O=O Water Oxoglutaric acid C5H8O5 2-Hydroxyglutarate 148.03717 Q9Y5U8 UniProt HMDB0002111 HMDB Reaction6224 false Isocitric acid + NADP ↔ NADPH + Oxoglutaric acid REVERSIBLE Reaction6225 false NADPH + Oxoglutaric acid → 2-Hydroxyglutarate + NADP LEFT_TO_RIGHT HMDB0001022 HMDB Adenosine diphosphate Reaction6226 false 2-Hydroxyglutarate + NADP ← NADPH + Oxoglutaric acid RIGHT_TO_LEFT 146-14-5 CAS 14265-44-2 CAS PYRUVATE BioCyc QH2 BioCyc 1.0 58-64-0 CAS Q9GZT9 UniProt Isocitric acid GTP BioCyc 585-84-2 CAS C00091 KEGG Compound 1.0 P11177 UniProt TransportCatalysis446 ACTIVATION HMDB0001273 HMDB Isocitric acid 444493 PubChem-compound ReactionCatalysis6154 ACTIVATION ReactionCatalysis6153 ACTIVATION Succinic acid ReactionCatalysis6152 ACTIVATION CHEBI:17084 ChEBI Dihydrolipoyllysine-residue succinyltransferase component of 2-oxoglutarate dehydrogenase complex, mitochondrial 22833512 PubChem-compound Isocitrate dehydrogenase [NADP] cytoplasmic ACETYL-COA BioCyc H2O Water 18.010565 1.0 ReactionCatalysis6158 ACTIVATION ReactionCatalysis6157 ACTIVATION FADH2 BioCyc Succinic acid HMDB0000193 HMDB Hydrogen carbonate HMDB0000072 HMDB ReactionCatalysis6155 ACTIVATION NADH BioCyc NAD BioCyc 977 PubChem-compound 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 Q99643 UniProt O75390 UniProt 146-91-8 CAS C5H9NO3 4-Hydroxyproline 131.05824 1.14.11.29 false 1.14.11.29 L-Proline + Oxoglutaric acid + Oxygen → 4-Hydroxyproline + Carbon dioxide + Succinic acid LEFT_TO_RIGHT Citrate synthase, mitochondrial C4H6O5 L-Malic acid 134.02153 1.0 CHO3 Hydrogen carbonate 60.99257 17215925 ChemSpider 446013 PubChem-compound C4H4O5 Oxalacetic acid 132.00587 L-Malic acid Succinyl-CoA ligase Adenosine triphosphate 1.0 Succinic acid 2.0 21883788 PubChem-compound Citric acid 1.0 SMILES O CIT BioCyc Guanosine triphosphate 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 SMILES O[C@H]1CN[C@@H](C1)C(O)=O 1.0 Q9UBX3 UniProt 6816 PubChem-compound 328-42-7 CAS 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 Succinate dehydrogenase cytochrome b560 subunit, mitochondrial CHEBI:46245 ChEBI 1.0 1.0 53-59-8 CAS C6H8O7 Isocitric acid 192.02701 1038 PubChem-compound Isocitrate dehydrogenase [NAD] subunit alpha, mitochondrial Isocitrate dehydrogenase [NADP] cytoplasmic Oxoglutarate dehydrogenase complex QH2 Isocitrate dehydrogenase [NAD] subunit gamma, mitochondrial GO:0005829 GENE ONTOLOGY Isocitrate dehydrogenase [NAD] subunit beta, mitochondrial Isocitrate dehydrogenase [NADP] cytoplasmic CHEBI:18095 ChEBI 274 ChemSpider C5H6O5 Oxoglutaric acid 146.02153 393487 ChemSpider Citric acid CHEBI:16908 ChEBI Pyruvate dehydrogenase complex Pyruvate dehydrogenase E1 component subunit beta, mitochondrial O4P Phosphate 94.95342 NADPH 6830 PubChem-compound 77-92-9 CAS 558863 ChemSpider SMP0002292 SMPDB 1.0 NADP C21H30N7O17P3 NADPH 745.0911 Mitochondrion C21H29N7O17P3 NADP 744.08325 NADPH BioCyc 643757 PubChem-compound Isocitrate dehydrogenase [NAD] subunit beta, mitochondrial HMDB0000208 HMDB CPD-6641 BioCyc 5810 PubChem-compound SubPathwayInteraction3389 SubPathway3389Reaction SubPathwayReaction 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 HMDB0000217 HMDB P48735 UniProt 124-38-9 CAS CHEBI:30915 ChEBI Q96HY7 UniProt 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 ReactionCatalysis4 ACTIVATION HMDB0001429 HMDB ReactionCatalysis63 ACTIVATION Mitochondrial dicarboxylate carrier ReactionCatalysis64 ACTIVATION HMDB0059655 HMDB ReactionCatalysis62 ACTIVATION ReactionCatalysis67 ACTIVATION 4.2.1.3 false 4.2.1.3 Citric acid → Isocitric acid LEFT_TO_RIGHT ReactionCatalysis68 ACTIVATION SMILES NC(=O)C1=C[N+](=CC=C1)[C@@H]1O[C@H](CO[P@](O)(=O)O[P@](O)(=O)OC[C@H]2O[C@H]([C@H](OP(O)(O)=O)[C@@H]2O)N2C=NC3=C(N)N=CN=C23)[C@@H](O)[C@H]1O HMDB0000221 HMDB 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 SMILES NC(=O)C1=CN(C=CC1)[C@H]1O[C@@H](COP(O)(=O)OP(O)(=O)OC[C@@H]2O[C@@H]([C@@H](OP(O)(O)=O)[C@H]2O)N2C=NC3=C(N)N=CN=C23)[C@H](O)[C@@H]1O Q96I99 UniProt Fumaric acid 1.0 1.0 C4H4O4 Fumaric acid 116.010956 P31040 UniProt Aconitate hydratase, mitochondrial 53-57-6 CAS Oxygen Egl nine homolog 2 CHEBI:15741 ChEBI Egl nine homolog 3 Fumarate hydratase, mitochondrial 1.0 Egl nine homolog 1 SMILES O=C=O SMILES OC(=O)[C@@H]1CCCN1 ReactionCatalysis78 ACTIVATION 4444052 ChemSpider 1110 PubChem-compound Hydrogen Ion 1.0 HMDB0001201 HMDB HMDB0000595 HMDB C21H29N7O14P2 NADH 665.12476 ReactionCatalysis70 ACTIVATION 5280344 PubChem-compound HMDB0001206 HMDB 5957 PubChem-compound P21399 UniProt 43 PubChem-compound GO:0005743 GENE ONTOLOGY Cytoplasmic aconitate hydratase 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 HMDB0000243 HMDB Citric acid 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 GO:0005737 GENE ONTOLOGY 1.0 147-85-3 CAS 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 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 Dihydrolipoyllysine-residue succinyltransferase component of 2-oxoglutarate dehydrogenase complex, mitochondrial 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 Egl nine homolog 3 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 Succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial Mitochondrial Matrix 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 222656 PubChem-compound 5675 ChemSpider 72-89-9 CAS 2.0 1078 ChemSpider C21H36N7O16P3S Coenzyme A 767.11523 Cytoplasm Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial CHEBI:15422 ChEBI Citrate synthase, mitochondrial 6557 ChemSpider 1.0 643975 PubChem-compound Homo sapiens Egl nine homolog 1 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 O2 Oxygen 31.98983 127-17-3 CAS SMILES OC(CCC(O)=O)C(O)=O P36957 UniProt Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex, mitochondrial Dihydrolipoyl dehydrogenase, mitochondrial CHEBI:16526 ChEBI Isocitrate dehydrogenase [NADP], mitochondrial 8977 PubChem-compound 1.0 1.0 CHEBI:16761 ChEBI NADP+-specific isocitrate dehydrogenase mutant Isocitrate dehydrogenase 2 [NADP], mitochondrial 7782-44-7 CAS 4.2.1.2 false 4.2.1.2 L-Malic acid ↔ Fumaric acid + Water REVERSIBLE Succinic acid 9606 TAXONOMY P08559 UniProt CHEBI:17544 ChEBI 2-KETOGLUTARATE BioCyc 392413 ChemSpider PW000146 PathWhiz HMDB0000725 HMDB 1.0 Oxoglutaric acid 8630 ChemSpider 5893 PubChem-compound FADH P10515 UniProt Succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial 110-15-6 CAS Citrate synthase, mitochondrial Oxoglutaric acid CHEBI:32816 ChEBI CHEBI:15377 ChEBI Fumarate hydratase, mitochondrial CHEBI:15379 ChEBI CHEBI:15378 ChEBI SMILES [H+] Q9H6Z9 UniProt 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 5886 PubChem-compound 51-35-4 CAS 60.0 CHEBI:17552 ChEBI Pyruvate carboxylase, mitochondrial L-4-HYDROXY-PROLINE BioCyc 280 PubChem-compound CHEBI:30744 ChEBI Dihydrolipoyl dehydrogenase, mitochondrial CHEBI:32805 ChEBI 1.0 Q9H302 UniProt C00417 KEGG Compound 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 Succinic acid FAD BioCyc 2.0 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 Egl nine homolog 2 Succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial SMP0000355 SMPDB 85-61-0 CAS L-Proline ReactionCatalysis2241 ACTIVATION UBIQUINONE-10 BioCyc 1.0 Malate dehydrogenase, mitochondrial Methylcytosine dioxygenase TET1 Lysine-specific demethylase 2A Hypoxia- inducible factor 1-alpha Mitochondrial pyruvate carrier 1 Mitochondrial dicarboxylate carrier 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 Pyruvate carboxylase, mitochondrial Aconitate hydratase, mitochondrial Aconitate hydratase, mitochondrial Isocitrate dehydrogenase [NADP] cytoplasmic NADP+-specific isocitrate dehydrogenase mutant Isocitrate dehydrogenase 2 [NADP], mitochondrial Isocitrate dehydrogenase [NADP], mitochondrial Succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial Succinate dehydrogenase cytochrome b560 subunit, mitochondrial Succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial Egl nine homolog 1 Egl nine homolog 2 Egl nine homolog 3 Pyruvic acid FADH 2 Pyruvic acid Succinic acid CoA NAD Acetyl-CoA CO 2 NADH H 2 O Oxalacetic acid Citric acid CoA Isocitric acid NAD Oxoglutaric acid CO 2 NADH H + NAD CoA Succinyl-CoA NADH H + CO 2 P i GDP CoA GTP L-Malic acid Fumaric acid H 2 O NAD NADH H + ATP HCO 3 - ADP P i cis-Aconitic acid H 2 O H 2 O Isocitric acid NADP Oxoglutaric acid NADPH NADPH 2-Hydroxyglutarate NADP NADP Oxoglutaric acid NADPH NADP Oxoglutaric acid NADPH Q FAD FADH 2 Fumaric acid QH 2 O 2 Oxoglutaric acid L-Proline CO 2 Succinic acid 4- Hydroxyproline Citric acid Oxygen Thiamine pyrophosphate Lipoamide FAD Magnesium Thiamine pyrophosphate Lipoamide FAD Biotin Manganese 4Fe-4S 4Fe-4S 4Fe-4S Magnesium Fe2+ Ascorbic acid Glycolysis Mitochondrial Electron Transport Chain Succinic acid Cytoplasmic aconitate hydratase Inner mitochondrial membrane Mitochondrial intermembrane space Cytosol Mitochondrial matrix Mitochondrion Intracellular Space Inner mitochondrial membrane Outer mitochondrial membrane Epigenetic Regulation Oncogene Activation, Tumour-suppressor inactivation Pseudo-Hypoxia Mutant SDH TCA cycle Citric acid Isocitric acid Oxoglutaric acid 2-Hydroxyglutarate Succinic acid Cytoplasmic aconitate hydratase Isocitrate dehydrogenase [NADP] cytoplasmic mutIDH1 prolyl hydroxylase Hypoxia-inducible factor 1-alpha Methylcytosine dioxygenase TET1 Lysine-specific demethylase 2A
5605 ChemSpider 1.0 6022 PubChem-compound C23H38N7O17P3S Acetyl-CoA 809.1258 PW002360 PathWhiz 439161 PubChem-compound Mitochondrial pyruvate carrier 1 937 ChemSpider C00311 KEGG Compound Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial CHEBI:30797 ChEBI 1.0 439153 PubChem-compound Mitochondrial dicarboxylate carrier 110-17-8 CAS Q96KS0 UniProt HMDB0000094 HMDB 50 ChemSpider 1.0 Succinyl-CoA ligase [GDP-forming] subunit beta, mitochondrial OXALACETIC_ACID BioCyc ATP BioCyc 1.0 Coenzyme Q10 FAD 4.0 HMDB0001072 HMDB 42 ChemSpider PRO BioCyc 1.0 HMDB0001197 HMDB Isocitrate dehydrogenase [NADP], mitochondrial mutant Isocitrate dehydrogenase 2 [NADP], mitochondrial PW000165 PathWhiz CHEBI:17203 ChEBI CHEBI:16238 ChEBI P09622 UniProt NADP+-specific isocitrate dehydrogenase HCO3 BioCyc CHEBI:15380 ChEBI 5742 ChemSpider C25H40N7O19P3S Succinyl-CoA 867.1312 1.0 CHEBI:16474 ChEBI C02630 KEGG Compound 952 ChemSpider 1.0 Carbon dioxide 1.0 1.0 5281915 PubChem-compound 97-67-6 CAS 2-Hydroxyglutarate C27H35N9O15P2 FADH 787.1728 Carbon dioxide 945 ChemSpider 1010 ChemSpider C10H15N5O11P2 Guanosine diphosphate 443.02432 O75874 UniProt 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 1.0 CHEBI:30769 ChEBI Succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial CHEBI:30887 ChEBI 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)NCCSC(=O)CCC(O)=O 1.0 C10H16N5O14P3 Guanosine triphosphate 522.99066 1161 ChemSpider Probable 2-oxoglutarate dehydrogenase E1 component DHKTD1, mitochondrial Cytosol SUC BioCyc O43837 UniProt Reaction390 false L-Malic acid + NAD ↔ Hydrogen Ion + NADH + Oxalacetic acid REVERSIBLE 1.0 C00009 KEGG Compound C00008 KEGG Compound C00007 KEGG Compound C00006 KEGG Compound CHEBI:18009 ChEBI 1.0 C00001 KEGG Compound C00122 KEGG Compound 559059 ChemSpider 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 C00005 KEGG Compound 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 C59H90O4 Coenzyme Q10 862.6839 Pyruvic acid SubPathwayOutput C27H33N9O15P2 FAD 785.1571 C21H28N7O14P2 NAD 664.11694 1.0 C00011 KEGG Compound C00010 KEGG Compound 1.0 Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial HMDB0000902 HMDB C00016 KEGG Compound 1.0 1.0 NAD(P) BioCyc FADH SubPathwayInput Pyruvate dehydrogenase E1 component subunit beta, mitochondrial H Hydrogen Ion 1.007825 C00149 KEGG Compound 388307 ChemSpider Phosphate C00022 KEGG Compound Pyruvate carboxylase, mitochondrial 56-65-5 CAS C01352 KEGG Compound 53-84-9 CAS C00148 KEGG Compound C00026 KEGG Compound C00024 KEGG Compound mutant succinate dehydrogenase Fumaric acid O14521 UniProt C00390 KEGG Compound 1.0 1.0 769 PubChem-compound The Oncogenic Action of Succinate Pyruvate carboxylase, mitochondrial 56275-39-9 CAS 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 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 328-50-7 CAS HMDB0000134 HMDB HMDB0000254 HMDB SMILES OC(=O)CCC(O)=O Transport517 false Succinic acid (→) Transport: Homo sapiens, Cell, Mitochondrial Inner Membrane to Homo sapiens, Cytosol LEFT_TO_RIGHT 1.0 P50213 UniProt 1.0 SMILES OC(=O)C\C(=C\C(O)=O)C(O)=O HMDB0001341 HMDB prolyl hydroxylase 4-Hydroxyproline P07954 UniProt 1.0 C00044 KEGG Compound 128566 ChemSpider C00042 KEGG Compound 4445197 ChemSpider C00288 KEGG Compound 303-98-0 CAS SMILES NC1=NC=NC2=C1N=CN2[C@@H]1O[C@H](COP(O)(=O)OP(O)(O)=O)[C@@H](O)[C@H]1O SMILES OC(=O)CC(O)(CC(O)=O)C(O)=O 604-98-8 CAS P51553 UniProt cis-Aconitic acid Succinyl-CoA 2889-31-8 CAS 10197150 ChemSpider FUM BioCyc SMILES OC(=O)CC(=O)C(O)=O 7732-18-5 CAS Aconitate hydratase, mitochondrial CHEBI:18012 ChEBI Cytoplasmic aconitate hydratase 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 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 SubPathwayInteraction3377 SubPathway3377Reaction SubPathwayReaction NAD 305 ChemSpider C01157 KEGG Compound HMDB0001377 HMDB SMILES CC(=O)C(O)=O Isocitrate dehydrogenase [NAD] subunit gamma, mitochondrial 1.0 Mitochondrial pyruvate carrier 1 6.4.1.1 false 6.4.1.1 Adenosine triphosphate + Hydrogen carbonate + Pyruvic acid → Adenosine diphosphate + Oxalacetic acid + Phosphate LEFT_TO_RIGHT HMDB0000162 HMDB 962 PubChem-compound 1.0 C5H9NO2 L-Proline 115.06333 5800 ChemSpider H0YLL5 UniProt CO2 Carbon dioxide 43.98983 145742 PubChem-compound 1.1.1.42 false 1.1.1.42 Isocitric acid + NADP → NADPH + Oxoglutaric acid LEFT_TO_RIGHT C00080 KEGG Compound 1.3.5.1 false 1.3.5.1 Coenzyme Q10 + FAD + Succinic acid ↔ FADH + Fumaric acid + QH2 REVERSIBLE SMILES O=O Water Oxoglutaric acid C5H8O5 2-Hydroxyglutarate 148.03717 Q9Y5U8 UniProt HMDB0002111 HMDB Reaction6224 false Isocitric acid + NADP ↔ NADPH + Oxoglutaric acid REVERSIBLE Reaction6225 false NADPH + Oxoglutaric acid → 2-Hydroxyglutarate + NADP LEFT_TO_RIGHT HMDB0001022 HMDB Adenosine diphosphate Reaction6226 false 2-Hydroxyglutarate + NADP ← NADPH + Oxoglutaric acid RIGHT_TO_LEFT 146-14-5 CAS 14265-44-2 CAS