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CHEBI:16015 ChEBI 6022 PubChem-compound Q9UI32 UniProt C23H38N7O17P3S Acetyl-CoA 809.1258 439161 PubChem-compound Mitochondrial pyruvate carrier 1 937 ChemSpider C00311 KEGG Compound CHEBI:16134 ChEBI Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial CHEBI:30797 ChEBI 439153 PubChem-compound 1.0 110-17-8 CAS HMDB0000094 HMDB 50 ChemSpider 1.0 1.0 Succinyl-CoA ligase [GDP-forming] subunit beta, mitochondrial NAD OXALACETIC_ACID BioCyc 439391 PubChem-compound ATP BioCyc 1.0 H4N Ammonium 18.034374 1.0 1.0 4.0 HMDB0001072 HMDB 42 ChemSpider HMDB0001197 HMDB 1.0 Isocitrate dehydrogenase [NADP], mitochondrial mutant Isocitrate dehydrogenase 2 [NADP], mitochondrial PW000165 PathWhiz CHEBI:16238 ChEBI P09622 UniProt 5746 ChemSpider NADP+-specific isocitrate dehydrogenase HCO3 BioCyc CHEBI:15380 ChEBI 5742 ChemSpider C25H40N7O19P3S Succinyl-CoA 867.1312 Succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial CHEBI:16474 ChEBI C02630 KEGG Compound 1.0 1.0 5281915 PubChem-compound 97-67-6 CAS 2-Hydroxyglutarate C27H35N9O15P2 FADH 787.1728 PW002452 PathWhiz 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 ReactionCatalysis6488 ACTIVATION ReactionCatalysis6489 ACTIVATION 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 Ammonium C10H16N5O14P3 Guanosine triphosphate 522.99066 Hydrogen Ion C5H10N2O3 L-Glutamine 146.06914 Q8N465 UniProt 1.0 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 C00006 KEGG Compound Succinate dehydrogenase 1.0 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 Succinate dehydrogenase cytochrome b560 subunit, mitochondrial C00005 KEGG Compound 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 C27H33N9O15P2 FAD 785.1571 Pyruvic acid SubPathwayOutput C21H28N7O14P2 NAD 664.11694 1.0 C00011 KEGG Compound C01342 KEGG Compound C00010 KEGG Compound Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial HMDB0000902 HMDB C00016 KEGG Compound FAD C00014 KEGG Compound 1.0 1.0 NAD(P) BioCyc FADH SubPathwayInput D-2-hydroxyglutarate dehydrogenase, mitochondrial Pyruvate dehydrogenase E1 component subunit beta, mitochondrial H Hydrogen Ion 1.007825 C00149 KEGG Compound 388307 ChemSpider Phosphate C00022 KEGG Compound L-Glutamine Pyruvate carboxylase, mitochondrial 56-65-5 CAS C01352 KEGG Compound 53-84-9 CAS C00026 KEGG Compound C00025 KEGG Compound C00024 KEGG Compound Fumaric acid O14521 UniProt C00390 KEGG Compound 1.0 13095-48-2 CAS 1.0 C5H8O5 L-2-Hydroxyglutaric acid 148.03717 769 PubChem-compound 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 Water P50213 UniProt 1.0 SMILES OC(=O)C\C(=C\C(O)=O)C(O)=O L-2-hydroxyglutarate dehydrogenase, mitochondrial HMDB0001341 HMDB 1.0 P07954 UniProt 1.0 C00044 KEGG Compound C00042 KEGG Compound 4445197 ChemSpider 3.5.1.2 false 3.5.1.2 L-Glutamine + Water → Ammonia + L-Glutamic acid LEFT_TO_RIGHT C00288 KEGG Compound 303-98-0 CAS 56-85-9 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 HMDB0000148 HMDB 13095-47-1 CAS P51553 UniProt cis-Aconitic acid Glutaminase liver isoform, mitochondrial Succinyl-CoA 2889-31-8 CAS 10197150 ChemSpider Reaction6570 false NADPH + Oxoglutaric acid → L-2-Hydroxyglutaric acid + NADP LEFT_TO_RIGHT 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 Reaction6569 false NADPH + Oxoglutaric acid → L-2-Hydroxyglutaric acid + NADP LEFT_TO_RIGHT Q99798 UniProt CHEBI:32797 ChEBI HMDB0001248 HMDB Pyruvic acid AMMONIA BioCyc SMILES N 56-86-0 CAS 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 C00064 KEGG Compound CHEBI:32796 ChEBI NAD 305 ChemSpider L-2-Hydroxyglutaric acid SMILES CC(=O)C(O)=O Isocitrate dehydrogenase [NAD] subunit gamma, mitochondrial 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 388508 ChemSpider Glutamate dehydrogenase 1, mitochondrial 962 PubChem-compound 1.0 5800 ChemSpider H0YLL5 UniProt ReactionCatalysis6492 ACTIVATION CO2 Carbon dioxide 43.98983 1.1.1.42 false 1.1.1.42 Isocitric acid + NADP → NADPH + Oxoglutaric acid LEFT_TO_RIGHT C00080 KEGG Compound Reaction6228 false Ammonium + Hydrogen Ion + NADPH + Oxoglutaric acid → L-Glutamic acid + NADP + Water LEFT_TO_RIGHT Water Oxoglutaric acid C5H8O5 2-Hydroxyglutarate 148.03717 HMDB0000051 HMDB Q9Y5U8 UniProt HMDB0002111 HMDB SMILES N[C@@H](CCC(O)=O)C(O)=O Reaction6223 false NADP + Oxoglutaric acid → 2-Hydroxyglutarate + NADPH LEFT_TO_RIGHT Reaction6224 false Isocitric acid + NADP ↔ NADPH + Oxoglutaric acid REVERSIBLE C5H8O5 D-2-Hydroxyglutaric acid 148.03717 HMDB0001022 HMDB Adenosine diphosphate 146-14-5 CAS 14265-44-2 CAS PYRUVATE BioCyc QH2 BioCyc 1.0 58-64-0 CAS Q9H9P8 UniProt Glutaminase liver isoform, mitochondrial Isocitric acid GTP BioCyc 585-84-2 CAS C00091 KEGG Compound 1.0 P11177 UniProt 388969 ChemSpider 1.1.99.- false 1.1.99.- NADH + Oxoglutaric acid → D-2-Hydroxyglutaric acid + NAD LEFT_TO_RIGHT HMDB0001273 HMDB Isocitric acid 444493 PubChem-compound 439939 PubChem-compound Succinic acid ReactionCatalysis6152 ACTIVATION ReactionCatalysis6151 ACTIVATION CHEBI:17084 ChEBI SubPathwayInteraction3687 SubPathwayReaction SubPathway3687Reaction Dihydrolipoyllysine-residue succinyltransferase component of 2-oxoglutarate dehydrogenase complex, mitochondrial 22833512 PubChem-compound D-2-Hydroxyglutaric acid Isocitrate dehydrogenase [NADP] cytoplasmic ACETYL-COA BioCyc CHEBI:18050 ChEBI H2O Water 18.010565 1.0 FADH2 BioCyc Succinic acid ReactionCatalysis6156 ACTIVATION HMDB0000193 HMDB Hydrogen carbonate HMDB0000072 HMDB ReactionCatalysis6155 ACTIVATION NADH BioCyc NAD BioCyc HMDB0059597 HMDB HMDB0041827 HMDB Succinyl-CoA ligase [ADP/GDP-forming] subunit alpha, mitochondrial Glutamate dehydrogenase 1, mitochondrial 749 ChemSpider 71-52-3 CAS SubPathwayInteraction3699 SubPathwayReaction SubPathway3699Reaction 970 PubChem-compound CIS-ACONITATE BioCyc SMILES OC(=O)\C=C\C(O)=O Acetyl-CoA Q99643 UniProt O75390 UniProt 146-91-8 CAS Citrate synthase, mitochondrial C4H6O5 L-Malic acid 134.02153 CHO3 Hydrogen carbonate 60.99257 30572 ChemSpider 17215925 ChemSpider 446013 PubChem-compound C4H4O5 Oxalacetic acid 132.00587 L-Malic acid Succinyl-CoA ligase Adenosine triphosphate 1.0 C01087 KEGG Compound NADH 2.0 21883788 PubChem-compound Citric acid 1.0 SMILES O CIT BioCyc Guanosine triphosphate H3N Ammonia 17.026548 Succinate dehydrogenase cytochrome b560 subunit, mitochondrial D-2-hydroxyglutarate dehydrogenase, mitochondrial P11498 UniProt HMDB0000538 HMDB Succinyl-CoA ligase [GDP-forming] subunit beta, mitochondrial (C5H8)nC14H20O4 QH2 5961 PubChem-compound TransportCatalysis6 ACTIVATION Succinyl-CoA ligase [ADP/GDP-forming] subunit alpha, mitochondrial 1.0 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 P00367 UniProt ADP BioCyc C3H4O3 Pyruvic acid 88.016045 CHEBI:46245 ChEBI 1.0 53-59-8 CAS C6H8O7 Isocitric acid 192.02701 SMILES O[C@@H](CCC(O)=O)C(O)=O 1038 PubChem-compound Isocitrate dehydrogenase [NAD] subunit alpha, mitochondrial Isocitrate dehydrogenase [NADP] cytoplasmic Oxoglutarate dehydrogenase complex Isocitrate dehydrogenase [NAD] subunit gamma, mitochondrial GO:0005829 GENE ONTOLOGY Isocitrate dehydrogenase [NAD] subunit beta, mitochondrial The Oncogenic Action of D-2-Hydroxyglutarate in Hydroxygluaricaciduria Ammonia Isocitrate dehydrogenase [NADP] cytoplasmic 274 ChemSpider C5H6O5 Oxoglutaric acid 146.02153 393487 ChemSpider Citric acid CHEBI:16908 ChEBI L-2-hydroxyglutarate dehydrogenase, mitochondrial Pyruvate dehydrogenase complex Pyruvate dehydrogenase E1 component subunit beta, mitochondrial O4P Phosphate 94.95342 NADPH 6830 PubChem-compound 77-92-9 CAS 558863 ChemSpider ReactionCatalysis43 ACTIVATION 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 218 ChemSpider 217 ChemSpider 58-68-4 CAS HMDB0000694 HMDB C03196 KEGG Compound SMP0000040 SMPDB HMDB0001423 HMDB Water L-Glutamic acid 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 1.0 SMP0002359 SMPDB HMDB0001429 HMDB ReactionCatalysis63 ACTIVATION 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 GLN BioCyc GLT BioCyc 7664-41-7 CAS 1.0 1.0 C4H4O4 Fumaric acid 116.010956 P31040 UniProt Aconitate hydratase, mitochondrial 53-57-6 CAS CHEBI:15741 ChEBI Fumarate hydratase, mitochondrial SMILES O=C=O ReactionCatalysis75 ACTIVATION CHEBI:28938 ChEBI ReactionCatalysis78 ACTIVATION 4444052 ChemSpider 222 PubChem-compound 1110 PubChem-compound Hydrogen Ion 1.0 HMDB0001201 HMDB HMDB0000595 HMDB SMILES O[C@H](CCC(O)=O)C(O)=O C21H29N7O14P2 NADH 665.12476 ReactionCatalysis70 ACTIVATION 5280344 PubChem-compound HMDB0001206 HMDB 5957 PubChem-compound P21399 UniProt GO:0005743 GENE ONTOLOGY 43 PubChem-compound 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 1.0 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 1.0 C6H8O7 Citric acid 192.02701 Probable 2-oxoglutarate dehydrogenase E1 component DHKTD1, mitochondrial 1.0 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 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 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 222656 PubChem-compound Fumaric acid 5675 ChemSpider 72-89-9 CAS 2.0 1078 ChemSpider 1.0 C21H36N7O16P3S Coenzyme A 767.11523 14798-03-9 CAS 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 SMILES OC(CCC(O)=O)C(O)=O C5H9NO4 L-Glutamic acid 147.05316 33032 PubChem-compound SMILES [NH4+] 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 1.0 CHEBI:16761 ChEBI NADP+-specific isocitrate dehydrogenase mutant Isocitrate dehydrogenase 2 [NADP], mitochondrial 4.2.1.2 false 4.2.1.2 L-Malic acid ↔ Fumaric acid + Water REVERSIBLE 9606 TAXONOMY P08559 UniProt CHEBI:17544 ChEBI 2-KETOGLUTARATE BioCyc 392413 ChemSpider 1.0 PW000146 PathWhiz HMDB0000606 HMDB Oxoglutaric acid 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+] 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 60.0 CHEBI:17552 ChEBI Pyruvate carboxylase, mitochondrial 1.0 1.0 280 PubChem-compound CHEBI:30744 ChEBI Dihydrolipoyl dehydrogenase, mitochondrial CHEBI:32805 ChEBI 1.0 Q9H302 UniProt C00417 KEGG Compound 16741146 PubChem-compound 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 SMILES N[C@@H](CCC(N)=O)C(O)=O 1198 PubChem-compound 1.0 1.0 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 HMDB0000641 HMDB SMP0000355 SMPDB 85-61-0 CAS UBIQUINONE-10 BioCyc ReactionCatalysis2241 ACTIVATION Malate dehydrogenase, mitochondrial Methylcytosine dioxygenase TET1 Lysine-specific demethylase 2A 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 Pyruvate carboxylase, mitochondrial Succinate dehydrogenase Aconitate hydratase, mitochondrial Aconitate hydratase, mitochondrial Glutamate dehydrogenase 1, mitochondrial Glutaminase liver isoform, mitochondrial Cytoplasmic aconitate hydratase Isocitrate dehydrogenase [NADP] cytoplasmic Isocitrate dehydrogenase [NADP], mitochondrial mutant Isocitrate dehydrogenase 2 [NADP], mitochondrial NADP+-specific isocitrate dehydrogenase D-2- hydroxyglutarate dehydrogenase, mitochondrial L-2- hydroxyglutarate dehydrogenase, mitochondrial Pyruvic acid FADH 2 Pyruvic 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 Succinic acid CoA GTP L-Malic acid Fumaric 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 NADPH Ammonium H + L-Glutamic acid H 2 O NADP L-Glutamine H 2 O NH 3 Isocitric acid NADP Oxoglutaric acid NADPH NADP Oxoglutaric acid NADPH NADPH NADP L-2-Hydroxyglutaric acid NADPH NADP L-2-Hydroxyglutaric acid NADH NAD NADP 2-Hydroxyglutarate NADPH Citric acid Thiamine pyrophosphate Lipoamide FAD Magnesium Thiamine pyrophosphate Lipoamide FAD Biotin Manganese 4Fe-4S 4Fe-4S 4Fe-4S Magnesium FAD Glycolysis Mitochondrial Electron Transport Chain D-2-Hydroxyglutaric acid Inner mitochondrial membrane Mitochondrial intermembrane space Cytosol Mitochondrial matrix Mitochondrion Intracellular Space Inner mitochondrial membrane Outer mitochondrial membrane Cytosol Epigenetic Regulation Oncogene Activation, Tumour-suppressor inactivation TCA cycle Citric acid Isocitric acid Oxoglutaric acid D-2-Hydroxyglutaric acid 2-Hydroxyglutarate Oxoglutaric acid Isocitrate dehydrogenase [NADP] cytoplasmic Cytoplasmic aconitase hydratase mutIDH2 D-2-hydroxyglutarate dehydrogenase, mitochondrial L-2-hydroxyglutarate dehydrogenase, mitochondrial Methylcytosine dioxygenase TET1 Lysine-specific demethylase 2A
CHEBI:16015 ChEBI 6022 PubChem-compound Q9UI32 UniProt C23H38N7O17P3S Acetyl-CoA 809.1258 439161 PubChem-compound Mitochondrial pyruvate carrier 1 937 ChemSpider C00311 KEGG Compound CHEBI:16134 ChEBI Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial CHEBI:30797 ChEBI 439153 PubChem-compound 1.0 110-17-8 CAS HMDB0000094 HMDB 50 ChemSpider 1.0 1.0 Succinyl-CoA ligase [GDP-forming] subunit beta, mitochondrial NAD OXALACETIC_ACID BioCyc 439391 PubChem-compound ATP BioCyc 1.0 H4N Ammonium 18.034374 1.0 1.0 4.0 HMDB0001072 HMDB 42 ChemSpider HMDB0001197 HMDB 1.0 Isocitrate dehydrogenase [NADP], mitochondrial mutant Isocitrate dehydrogenase 2 [NADP], mitochondrial PW000165 PathWhiz CHEBI:16238 ChEBI P09622 UniProt 5746 ChemSpider NADP+-specific isocitrate dehydrogenase HCO3 BioCyc CHEBI:15380 ChEBI 5742 ChemSpider C25H40N7O19P3S Succinyl-CoA 867.1312 Succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial CHEBI:16474 ChEBI C02630 KEGG Compound 1.0 1.0 5281915 PubChem-compound 97-67-6 CAS 2-Hydroxyglutarate C27H35N9O15P2 FADH 787.1728 PW002452 PathWhiz 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 ReactionCatalysis6488 ACTIVATION ReactionCatalysis6489 ACTIVATION 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 Ammonium C10H16N5O14P3 Guanosine triphosphate 522.99066 Hydrogen Ion C5H10N2O3 L-Glutamine 146.06914 Q8N465 UniProt 1.0 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 C00006 KEGG Compound Succinate dehydrogenase 1.0 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 Succinate dehydrogenase cytochrome b560 subunit, mitochondrial C00005 KEGG Compound 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 C27H33N9O15P2 FAD 785.1571 Pyruvic acid SubPathwayOutput C21H28N7O14P2 NAD 664.11694 1.0 C00011 KEGG Compound C01342 KEGG Compound C00010 KEGG Compound Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial HMDB0000902 HMDB C00016 KEGG Compound FAD C00014 KEGG Compound 1.0 1.0 NAD(P) BioCyc FADH SubPathwayInput D-2-hydroxyglutarate dehydrogenase, mitochondrial Pyruvate dehydrogenase E1 component subunit beta, mitochondrial H Hydrogen Ion 1.007825 C00149 KEGG Compound 388307 ChemSpider Phosphate C00022 KEGG Compound L-Glutamine Pyruvate carboxylase, mitochondrial 56-65-5 CAS C01352 KEGG Compound 53-84-9 CAS C00026 KEGG Compound C00025 KEGG Compound C00024 KEGG Compound Fumaric acid O14521 UniProt C00390 KEGG Compound 1.0 13095-48-2 CAS 1.0 C5H8O5 L-2-Hydroxyglutaric acid 148.03717 769 PubChem-compound 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 Water P50213 UniProt 1.0 SMILES OC(=O)C\C(=C\C(O)=O)C(O)=O L-2-hydroxyglutarate dehydrogenase, mitochondrial HMDB0001341 HMDB 1.0 P07954 UniProt 1.0 C00044 KEGG Compound C00042 KEGG Compound 4445197 ChemSpider 3.5.1.2 false 3.5.1.2 L-Glutamine + Water → Ammonia + L-Glutamic acid LEFT_TO_RIGHT C00288 KEGG Compound 303-98-0 CAS 56-85-9 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 HMDB0000148 HMDB 13095-47-1 CAS P51553 UniProt cis-Aconitic acid Glutaminase liver isoform, mitochondrial Succinyl-CoA 2889-31-8 CAS 10197150 ChemSpider Reaction6570 false NADPH + Oxoglutaric acid → L-2-Hydroxyglutaric acid + NADP LEFT_TO_RIGHT 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 Reaction6569 false NADPH + Oxoglutaric acid → L-2-Hydroxyglutaric acid + NADP LEFT_TO_RIGHT Q99798 UniProt CHEBI:32797 ChEBI HMDB0001248 HMDB Pyruvic acid AMMONIA BioCyc SMILES N 56-86-0 CAS 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 C00064 KEGG Compound CHEBI:32796 ChEBI NAD 305 ChemSpider L-2-Hydroxyglutaric acid SMILES CC(=O)C(O)=O Isocitrate dehydrogenase [NAD] subunit gamma, mitochondrial 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 388508 ChemSpider Glutamate dehydrogenase 1, mitochondrial 962 PubChem-compound 1.0 5800 ChemSpider H0YLL5 UniProt ReactionCatalysis6492 ACTIVATION CO2 Carbon dioxide 43.98983 1.1.1.42 false 1.1.1.42 Isocitric acid + NADP → NADPH + Oxoglutaric acid LEFT_TO_RIGHT C00080 KEGG Compound Reaction6228 false Ammonium + Hydrogen Ion + NADPH + Oxoglutaric acid → L-Glutamic acid + NADP + Water LEFT_TO_RIGHT Water Oxoglutaric acid C5H8O5 2-Hydroxyglutarate 148.03717 HMDB0000051 HMDB Q9Y5U8 UniProt HMDB0002111 HMDB SMILES N[C@@H](CCC(O)=O)C(O)=O Reaction6223 false NADP + Oxoglutaric acid → 2-Hydroxyglutarate + NADPH LEFT_TO_RIGHT Reaction6224 false Isocitric acid + NADP ↔ NADPH + Oxoglutaric acid REVERSIBLE C5H8O5 D-2-Hydroxyglutaric acid 148.03717 HMDB0001022 HMDB Adenosine diphosphate 146-14-5 CAS 14265-44-2 CAS PYRUVATE BioCyc QH2 BioCyc 1.0 58-64-0 CAS Q9H9P8 UniProt Glutaminase liver isoform, mitochondrial Isocitric acid GTP BioCyc 585-84-2 CAS C00091 KEGG Compound