Label Type Identifier Comment Ensembl NCBI gene HGNC UniProt Wikidata ChEBI InChI Alanine Metabolite chebi:16977 "" wikidata:Q218642 chebi:16977;chebi:16977 inchikey:QNAYBMKLOCPYGJ-REOHCLBHSA-N Alpha-ketoglutarate Metabolite chebi:16810 "" wikidata:Q27102088 chebi:16810;chebi:16810 inchikey:KPGXRSRHYNQIFN-UHFFFAOYSA-L Fumarate Metabolite chebi:18012 "" wikidata:Q139857 chebi:18012;chebi:18012 inchikey:VZCYOOQTPOCHFL-OWOJBTEDSA-N Oxaloacetate Metabolite wikidata:Q408658 "Not in original drawing, adapted from https://www.wikipathways.org/index.php/Pathway:WP78" wikidata:Q408658 chebi:30744;chebi:30744 inchikey:KHPXUQMNIQBQEV-UHFFFAOYSA-N Methylmalonyl-CoA Metabolite hmdb:HMDB0001269 "" chebi:15466;chebi:15466 inchikey:MZFOKIKEPGUZEN-PNEPRAIJSA-N (S)-malate Metabolite wikidata:Q27104150 "Not in original drawing, adapted from https://www.wikipathways.org/index.php/Pathway:WP78" wikidata:Q27104150 chebi:30797;chebi:30797 inchikey:BJEPYKJPYRNKOW-REOHCLBHSA-N Pyruvate Metabolite chebi:15361 "" wikidata:Q27089397 chebi:15361;chebi:15361 inchikey:LCTONWCANYUPML-UHFFFAOYSA-M Lactate Metabolite chebi:24996 "" wikidata:Q27895931 chebi:24996;chebi:24996 inchikey:JVTAAEKCZFNVCJ-UHFFFAOYSA-M Succinyl coenzyme A Metabolite chebi:15380 "" wikidata:Q419560 chebi:15380;chebi:15380 inchikey:VNOYUJKHFWYWIR-ITIYDSSPSA-N Acetyl-CoA Metabolite wikidata:Q715317 "" wikidata:Q715317 chebi:15351;chebi:15351 inchikey:ZSLZBFCDCINBPY-ZSJPKINUSA-N ADP Metabolite chebi:16761 "" wikidata:Q185253 chebi:16761;chebi:16761 inchikey:XTWYTFMLZFPYCI-KQYNXXCUSA-N Propionyl Metabolite chebi:26294 "" wikidata:Q76592161 chebi:26294;chebi:26294 ATP Metabolite chebi:15422 "" wikidata:Q80863 chebi:15422;chebi:15422 inchikey:ZKHQWZAMYRWXGA-KQYNXXCUSA-N Methylmalonicacid Metabolite chebi:30860 "" wikidata:Q239598 chebi:30860;chebi:30860 inchikey:ZIYVHBGGAOATLY-UHFFFAOYSA-N GTP Metabolite chebi:15996 "" wikidata:Q392227 chebi:15996;chebi:15996 inchikey:XKMLYUALXHKNFT-UUOKFMHZSA-N Citrate Metabolite chebi:133748 "" wikidata:Q55502846 chebi:133748;chebi:133748 Succinate Metabolite chebi:30031 "" wikidata:Q27104100 chebi:30031;chebi:30031 inchikey:KDYFGRWQOYBRFD-UHFFFAOYSA-L Methylcitrate Metabolite wikidata:Q27104155 "a.k.a. 2-methylcitric acid" wikidata:Q27104155 chebi:30835;chebi:30835 inchikey:YNOXCRMFGMSKIJ-UHFFFAOYSA-N Methylmalonyl carnitineester (C4DC) Metabolite hmdb:HMDB0062785 "" chebi:85473;chebi:85473 inchikey:XROYFEWIXXCPAW-BRFYHDHCSA-N Succinyl carnitineester (C4DC) Metabolite wikidata:Q27140249 "" wikidata:Q27140249 chebi:73034;chebi:73034 inchikey:HAEVNYBCYZZDFL-UHFFFAOYSA-N 3-hydroxypropionate Metabolite wikidata:Q27101949 "" wikidata:Q27101949 chebi:16510;chebi:16510 inchikey:ALRHLSYJTWAHJZ-UHFFFAOYSA-M Propionylcarnitine (C3) Metabolite wikidata:Q27103933 "a.k.a. 2-methylcitric acid" wikidata:Q27103933 chebi:28867;chebi:28867 inchikey:UFAHZIUFPNSHSL-UHFFFAOYSA-N GDP Metabolite chebi:17552 "" wikidata:Q422582 chebi:17552;chebi:17552 inchikey:QGWNDRXFNXRZMB-UUOKFMHZSA-N Pyruvate Metabolite chebi:15361 "" wikidata:Q27089397 chebi:15361;chebi:15361 inchikey:LCTONWCANYUPML-UHFFFAOYSA-M Alanine Metabolite chebi:16977 "" wikidata:Q218642 chebi:16977;chebi:16977 inchikey:QNAYBMKLOCPYGJ-REOHCLBHSA-N FH GeneProduct ncbigene:2271 "Fumarate hydratase" ensembl:ENSG00000091483 ncbigene:2271 hgnc.symbol:FH uniprot:A0A804HI24;uniprot:A0A804HKE6;uniprot:P07954;uniprot:A0A804HL52;uniprot:A0A0S2Z4C3 NDPK Protein eccode:2.7.4.6 "Nucleoside diphosphate kinase" LDH Protein eccode:1.1.1.27 "AKA LDH lactate dehydrogenase" SUCLA2 Protein uniprot:Q9P2R7 "" ensembl:ENSG00000136143 ncbigene:8803 hgnc.symbol:SUCLA2 uniprot:Q5T9Q5;uniprot:A0A0U1RQU7;uniprot:Q5T9Q8;uniprot:A0A0U1RQL1;uniprot:A0A2R8YF84;uniprot:A0A2R8Y6E6;uniprot:A0A0U1RRI1;uniprot:E5KS60;uniprot:A0A0U1RQF8;uniprot:A0A2R8YDQ9;uniprot:A0A2R8Y6Y7;uniprot:A0A2R8Y5P6;uniprot:Q9P2R7;uniprot:A0A2R8Y5P4 PDH Protein eccode:1.2.4.1 "AKA pyruvate dehydrogenase" MPC Protein uniprot:Q9Y5U8 "AKA Mitochondrial pyruvate transport" ensembl:ENSG00000060762 ncbigene:51660 hgnc.symbol:MPC1 uniprot:A0A087WVZ0;uniprot:Q5TI65;uniprot:Q9Y5U8 ALT Protein uniprot:P24298 "AKA alanine aminotransferase" ensembl:ENSG00000167701 ncbigene:2875 hgnc.symbol:GPT uniprot:P24298 ADP-forming (A-SCS) Protein eccode:6.2.1.5 "SCS (succinyl-CoA syntethase)
For more info see: https://en.wikipedia.org/wiki/Succinyl_coenzyme_A_synthetase" LDHB Protein uniprot:P07195 "" ensembl:ENSG00000111716 ncbigene:3945 hgnc.symbol:LDHB uniprot:P07195;uniprot:Q5U077;uniprot:F5H793;uniprot:A0A5F9ZHM4;uniprot:A8MW50;uniprot:C9J7H8 PEPCK Protein ncbigene:5106 "AKA phosphoenolpyruvate carboxykinase" ensembl:ENSG00000100889 ncbigene:5106 hgnc.symbol:PCK2 uniprot:H0YNH9;uniprot:H0YMU6;uniprot:H0YMA5;uniprot:H0YM31;uniprot:H0YNG4;uniprot:Q16822;uniprot:A0A384MTT2;uniprot:H0YKC4;uniprot:H0YMY3;uniprot:H0YML5 LDHC Protein uniprot:P07864 "AKA lactate dehydrogenase C; expressed in testes." ensembl:ENSG00000166796 ncbigene:3948 hgnc.symbol:LDHC uniprot:F5GWS6;uniprot:F5GZ11;uniprot:F5H155;uniprot:A0A140VKA7;uniprot:F5H5G7;uniprot:P07864;uniprot:F5H245;uniprot:G3XAP5;uniprot:F5H326 E1-subunit Protein uniprot:Q96HY7 "" ensembl:ENSG00000181192 ncbigene:55526 hgnc.symbol:DHTKD1 uniprot:H7C1J3;uniprot:H7C149;uniprot:C9JWN1;uniprot:Q96HY7 GDP-forming (G-SCS) Protein eccode:6.2.1.4 "SCS (succinyl-CoA syntethase)
For more info see: https://en.wikipedia.org/wiki/Succinyl_coenzyme_A_synthetase" 2-KGD complex Protein uniprot:Q1B4V6 "Composed of 3 subunits
'Homodimer. The 2-oxoglutarate dehydrogenase (ODH) complex contains multiple copies of three enzymatic components: 2-oxoglutarate dehydrogenase (E1), dihydrolipoamide succinyltransferase (E2) and lipoamide dehydrogenase (E3) (By similarity).' [https://www.uniprot.org/uniprot/Q1B4V6]" E2-subunit Protein uniprot:P36957 "" ensembl:ENSG00000119689 ncbigene:1743 hgnc.symbol:DLST uniprot:A0A024R6C9;uniprot:G3V5M3;uniprot:G3V3F0;uniprot:G3V5L9;uniprot:H0YJF9;uniprot:G3V498;uniprot:P36957;uniprot:B7ZAZ8;uniprot:Q86SW4 E3-subunit Protein uniprot:O00330 "" ensembl:ENSG00000110435 ncbigene:8050 hgnc.symbol:PDHX uniprot:E9PLU0;uniprot:A0A8C8MSB2;uniprot:E9PRI6;uniprot:O00330;uniprot:H0YD97 SUCLG1 Protein uniprot:P53597 "Succinate-CoA ligase alpha-subunit" ensembl:ENSG00000163541 ncbigene:8802 hgnc.symbol:SUCLG1 uniprot:A0A494C0D1;uniprot:H7C233;uniprot:P53597 SUCLG2 Protein uniprot:Q96I99 "" ensembl:ENSG00000172340 ncbigene:8801 hgnc.symbol:SUCLG2 uniprot:Q96I99;uniprot:E9PDQ8;uniprot:H0Y852 SUCLG1 Protein uniprot:P53597 "" ensembl:ENSG00000163541 ncbigene:8802 hgnc.symbol:SUCLG1 uniprot:A0A494C0D1;uniprot:H7C233;uniprot:P53597 GDP-forming (G-SCS) Protein eccode:6.2.1.4 "SCS (succinyl-CoA syntethase)
For more info see: https://en.wikipedia.org/wiki/Succinyl_coenzyme_A_synthetase" SUCLG1 Protein uniprot:P53597 "Succinate-CoA ligase alpha-subunit" ensembl:ENSG00000163541 ncbigene:8802 hgnc.symbol:SUCLG1 uniprot:A0A494C0D1;uniprot:H7C233;uniprot:P53597 SUCLG2 Protein uniprot:Q96I99 "" ensembl:ENSG00000172340 ncbigene:8801 hgnc.symbol:SUCLG2 uniprot:Q96I99;uniprot:E9PDQ8;uniprot:H0Y852 SUCLA2 Protein uniprot:Q9P2R7 "" ensembl:ENSG00000136143 ncbigene:8803 hgnc.symbol:SUCLA2 uniprot:Q5T9Q5;uniprot:A0A0U1RQU7;uniprot:Q5T9Q8;uniprot:A0A0U1RQL1;uniprot:A0A2R8YF84;uniprot:A0A2R8Y6E6;uniprot:A0A0U1RRI1;uniprot:E5KS60;uniprot:A0A0U1RQF8;uniprot:A0A2R8YDQ9;uniprot:A0A2R8Y6Y7;uniprot:A0A2R8Y5P6;uniprot:Q9P2R7;uniprot:A0A2R8Y5P4 ADP-forming (A-SCS) Protein eccode:6.2.1.5 "SCS (succinyl-CoA syntethase)
For more info see: https://en.wikipedia.org/wiki/Succinyl_coenzyme_A_synthetase" SUCLG1 Protein uniprot:P53597 "" ensembl:ENSG00000163541 ncbigene:8802 hgnc.symbol:SUCLG1 uniprot:A0A494C0D1;uniprot:H7C233;uniprot:P53597 GDP-forming (G-SCS) Protein eccode:6.2.1.4 "SCS (succinyl-CoA syntethase)
For more info see: https://en.wikipedia.org/wiki/Succinyl_coenzyme_A_synthetase" SUCLG1 Protein uniprot:P53597 "Succinate-CoA ligase alpha-subunit" ensembl:ENSG00000163541 ncbigene:8802 hgnc.symbol:SUCLG1 uniprot:A0A494C0D1;uniprot:H7C233;uniprot:P53597 SUCLG2 Protein uniprot:Q96I99 "" ensembl:ENSG00000172340 ncbigene:8801 hgnc.symbol:SUCLG2 uniprot:Q96I99;uniprot:E9PDQ8;uniprot:H0Y852 NDPK Protein eccode:2.7.4.6 "Nucleoside diphosphate kinase" SUCLA2 Protein uniprot:Q9P2R7 "" ensembl:ENSG00000136143 ncbigene:8803 hgnc.symbol:SUCLA2 uniprot:Q5T9Q5;uniprot:A0A0U1RQU7;uniprot:Q5T9Q8;uniprot:A0A0U1RQL1;uniprot:A0A2R8YF84;uniprot:A0A2R8Y6E6;uniprot:A0A0U1RRI1;uniprot:E5KS60;uniprot:A0A0U1RQF8;uniprot:A0A2R8YDQ9;uniprot:A0A2R8Y6Y7;uniprot:A0A2R8Y5P6;uniprot:Q9P2R7;uniprot:A0A2R8Y5P4 ADP-forming (A-SCS) Protein eccode:6.2.1.5 "SCS (succinyl-CoA syntethase)
For more info see: https://en.wikipedia.org/wiki/Succinyl_coenzyme_A_synthetase" SUCLG1 Protein uniprot:P53597 "" ensembl:ENSG00000163541 ncbigene:8802 hgnc.symbol:SUCLG1 uniprot:A0A494C0D1;uniprot:H7C233;uniprot:P53597 NDPK Protein eccode:2.7.4.6 "Nucleoside diphosphate kinase" LDH1 Protein eccode:1.1.1.27 "AKA LDH-1 lactate dehydrogenase 1, 4H; expressed in heart, red blood cells (RBC), brain)" LDH2 Protein eccode:1.1.1.27 "AKA LDH-2 lactate dehydrogenase 2 (3H1M); expressed in reticuloendohelial system" LDH3 Protein eccode:1.1.1.27 "AKA LDH-3 lactate dehydrogenase 3 (2H2M); expressed in lungs" LDH4 Protein eccode:1.1.1.27 "AKA LDH-4 lactate dehydrogenase 4 (1H3M); expressed in kidneys, placenta, pancreas." LDH5 Protein eccode:1.1.1.27 "AKA LDH-5 lactate dehydrogenase 5 (4M); expressed in liver, striated muscle, brain." LDHB Protein uniprot:P07195 "" ensembl:ENSG00000111716 ncbigene:3945 hgnc.symbol:LDHB uniprot:P07195;uniprot:Q5U077;uniprot:F5H793;uniprot:A0A5F9ZHM4;uniprot:A8MW50;uniprot:C9J7H8 LDHB Protein uniprot:P07195 "" ensembl:ENSG00000111716 ncbigene:3945 hgnc.symbol:LDHB uniprot:P07195;uniprot:Q5U077;uniprot:F5H793;uniprot:A0A5F9ZHM4;uniprot:A8MW50;uniprot:C9J7H8 LDHB Protein uniprot:P07195 "" ensembl:ENSG00000111716 ncbigene:3945 hgnc.symbol:LDHB uniprot:P07195;uniprot:Q5U077;uniprot:F5H793;uniprot:A0A5F9ZHM4;uniprot:A8MW50;uniprot:C9J7H8 LDHB Protein uniprot:P07195 "" ensembl:ENSG00000111716 ncbigene:3945 hgnc.symbol:LDHB uniprot:P07195;uniprot:Q5U077;uniprot:F5H793;uniprot:A0A5F9ZHM4;uniprot:A8MW50;uniprot:C9J7H8 LDHB Protein uniprot:P07195 "" ensembl:ENSG00000111716 ncbigene:3945 hgnc.symbol:LDHB uniprot:P07195;uniprot:Q5U077;uniprot:F5H793;uniprot:A0A5F9ZHM4;uniprot:A8MW50;uniprot:C9J7H8 LDHB Protein uniprot:P07195 "" ensembl:ENSG00000111716 ncbigene:3945 hgnc.symbol:LDHB uniprot:P07195;uniprot:Q5U077;uniprot:F5H793;uniprot:A0A5F9ZHM4;uniprot:A8MW50;uniprot:C9J7H8 LDHA Protein uniprot:P00338 "" ensembl:ENSG00000134333 ncbigene:3939 hgnc.symbol:LDHA uniprot:F5GXC7;uniprot:V9HWB9;uniprot:F5GZQ4;uniprot:P00338;uniprot:F5H5J4;uniprot:F5GXU1;uniprot:F5GXY2;uniprot:F5GWW2;uniprot:F5GXH2;uniprot:F5H8H6;uniprot:F5GYU2;uniprot:F5H6W8 LDHB Protein uniprot:P07195 "" ensembl:ENSG00000111716 ncbigene:3945 hgnc.symbol:LDHB uniprot:P07195;uniprot:Q5U077;uniprot:F5H793;uniprot:A0A5F9ZHM4;uniprot:A8MW50;uniprot:C9J7H8 LDHB Protein uniprot:P07195 "" ensembl:ENSG00000111716 ncbigene:3945 hgnc.symbol:LDHB uniprot:P07195;uniprot:Q5U077;uniprot:F5H793;uniprot:A0A5F9ZHM4;uniprot:A8MW50;uniprot:C9J7H8 LDHA Protein uniprot:P00338 "" ensembl:ENSG00000134333 ncbigene:3939 hgnc.symbol:LDHA uniprot:F5GXC7;uniprot:V9HWB9;uniprot:F5GZQ4;uniprot:P00338;uniprot:F5H5J4;uniprot:F5GXU1;uniprot:F5GXY2;uniprot:F5GWW2;uniprot:F5GXH2;uniprot:F5H8H6;uniprot:F5GYU2;uniprot:F5H6W8 LDHA Protein uniprot:P00338 "" ensembl:ENSG00000134333 ncbigene:3939 hgnc.symbol:LDHA uniprot:F5GXC7;uniprot:V9HWB9;uniprot:F5GZQ4;uniprot:P00338;uniprot:F5H5J4;uniprot:F5GXU1;uniprot:F5GXY2;uniprot:F5GWW2;uniprot:F5GXH2;uniprot:F5H8H6;uniprot:F5GYU2;uniprot:F5H6W8 LDHB Protein uniprot:P07195 "" ensembl:ENSG00000111716 ncbigene:3945 hgnc.symbol:LDHB uniprot:P07195;uniprot:Q5U077;uniprot:F5H793;uniprot:A0A5F9ZHM4;uniprot:A8MW50;uniprot:C9J7H8 LDHA Protein uniprot:P00338 "" ensembl:ENSG00000134333 ncbigene:3939 hgnc.symbol:LDHA uniprot:F5GXC7;uniprot:V9HWB9;uniprot:F5GZQ4;uniprot:P00338;uniprot:F5H5J4;uniprot:F5GXU1;uniprot:F5GXY2;uniprot:F5GWW2;uniprot:F5GXH2;uniprot:F5H8H6;uniprot:F5GYU2;uniprot:F5H6W8 LDHA Protein uniprot:P00338 "" ensembl:ENSG00000134333 ncbigene:3939 hgnc.symbol:LDHA uniprot:F5GXC7;uniprot:V9HWB9;uniprot:F5GZQ4;uniprot:P00338;uniprot:F5H5J4;uniprot:F5GXU1;uniprot:F5GXY2;uniprot:F5GWW2;uniprot:F5GXH2;uniprot:F5H8H6;uniprot:F5GYU2;uniprot:F5H6W8 LDHA Protein uniprot:P00338 "" ensembl:ENSG00000134333 ncbigene:3939 hgnc.symbol:LDHA uniprot:F5GXC7;uniprot:V9HWB9;uniprot:F5GZQ4;uniprot:P00338;uniprot:F5H5J4;uniprot:F5GXU1;uniprot:F5GXY2;uniprot:F5GWW2;uniprot:F5GXH2;uniprot:F5H8H6;uniprot:F5GYU2;uniprot:F5H6W8 LDHA Protein uniprot:P00338 "" ensembl:ENSG00000134333 ncbigene:3939 hgnc.symbol:LDHA uniprot:F5GXC7;uniprot:V9HWB9;uniprot:F5GZQ4;uniprot:P00338;uniprot:F5H5J4;uniprot:F5GXU1;uniprot:F5GXY2;uniprot:F5GWW2;uniprot:F5GXH2;uniprot:F5H8H6;uniprot:F5GYU2;uniprot:F5H6W8 LDHA Protein uniprot:P00338 "" ensembl:ENSG00000134333 ncbigene:3939 hgnc.symbol:LDHA uniprot:F5GXC7;uniprot:V9HWB9;uniprot:F5GZQ4;uniprot:P00338;uniprot:F5H5J4;uniprot:F5GXU1;uniprot:F5GXY2;uniprot:F5GWW2;uniprot:F5GXH2;uniprot:F5H8H6;uniprot:F5GYU2;uniprot:F5H6W8 LDHA Protein uniprot:P00338 "" ensembl:ENSG00000134333 ncbigene:3939 hgnc.symbol:LDHA uniprot:F5GXC7;uniprot:V9HWB9;uniprot:F5GZQ4;uniprot:P00338;uniprot:F5H5J4;uniprot:F5GXU1;uniprot:F5GXY2;uniprot:F5GWW2;uniprot:F5GXH2;uniprot:F5H8H6;uniprot:F5GYU2;uniprot:F5H6W8 LDHA Protein uniprot:P00338 "" ensembl:ENSG00000134333 ncbigene:3939 hgnc.symbol:LDHA uniprot:F5GXC7;uniprot:V9HWB9;uniprot:F5GZQ4;uniprot:P00338;uniprot:F5H5J4;uniprot:F5GXU1;uniprot:F5GXY2;uniprot:F5GWW2;uniprot:F5GXH2;uniprot:F5H8H6;uniprot:F5GYU2;uniprot:F5H6W8 ALT Protein uniprot:P24298 "AKA alanine aminotransferase" ensembl:ENSG00000167701 ncbigene:2875 hgnc.symbol:GPT uniprot:P24298