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Malate dehydrogenase, mitochondrial Methylcytosine dioxygenase TET1 Lysine-specific demethylase 2A Mitochondrial pyruvate carrier 1 Mitochondrial citrate transport protein 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 NADP-specific glutamate dehydrogenase Glutaminase liver isoform, mitochondrial Isocitrate dehydrogenase [NADP] cytoplasmic NADP+-specific isocitrate dehydrogenase Isocitrate dehydrogenase [NADP], mitochondrial Cytoplasmic aconitate hydratase L-2- hydroxyglutarate dehydrogenase, mitochondrial Pyruvic acid FADH2 Pyruvic acid Citric acid Citric acid CoA NAD Acetyl-CoA CO2 NADH H2O Oxalacetic acid CoA Isocitric acid NAD Oxoglutaric acid CO2 NADH H+ NAD CoA Succinyl-CoA NADH H+ CO2 Pi GDP Succinic acid CoA GTP L-Malic acid Fumaric acid H2O NAD NADH H+ ATP HCO3- ADP Pi Q FAD QH2 cis-Aconitic acid H2O H2O NADPH Ammonium H+ L-Glutamic acid H2O NADP L-Glutamine H2O NH3 Isocitric acid NADP Oxoglutaric acid NADPH NADPH 2-Hydroxyglutarate NADP NADP Oxoglutaric acid NADPH NADP NADPH Thiamine pyrophosphate Lipoamide FAD Thiamine pyrophosphate Lipoamide FAD Biotin Manganese FAD Glycolysis Mitochondrial Electron Transport Chain 4Fe-4S 4Fe-4S 4Fe-4S Magnesium Magnesium Mitochondrial intermembrane space Mitochondrial matrix Mitochondrion Inner mitochondrial membrane Outer mitochondrial membrane Cytosol Epigenetic regulation: activation of enzymes that catalyze DNA demethylation. Oncogene activation Tumour-suppressor inactivation TCA cycle 2-Hydroxyglutarate mutIDH1 Methylcystosine dioxygenase TET1 Lysine-specific demethylase 2A Altered protein synthesis (oncogenic) Angiogenesis Cell proliferation
Mitochondria MDH2 TET1 KDM2A MPC1 Unknown PDHA1 CS IDH3A IDH3B IDH3G OGDH SUCLG1 SUCLG2 FH PC SDHA SDHB SDHC SDHD ACO2 ACO2 gdhA GLS2 IDH1 IDH1 IDH2 ACO1 L2HGDH Pyruvic acid FADH Pyruvic acid Citric acid Citric acid Coenzyme A NAD Acetyl-CoA Carbon dioxide NADH Water Oxalacetic acid Coenzyme A Isocitric acid NAD Oxoglutaric acid Carbon dioxide NADH Hydrogen Ion NAD Coenzyme A Succinyl-CoA NADH Hydrogen Ion Carbon dioxide Phosphate Guanosine diphosphate Succinic acid Coenzyme A Guanosine triphosphate L-Malic acid Fumaric acid Water NAD NADH Hydrogen Ion Adenosine triphosphate Hydrogen carbonate Adenosine diphosphate Phosphate Coenzyme Q10 FAD QH(2) cis-Aconitic acid Water Water NADPH Ammonium Hydrogen Ion L-Glutamic acid Water NADP L-Glutamine Water Ammonia Isocitric acid NADP Oxoglutaric acid NADPH NADPH 2- Hydroxyglutarate NADP NADP Oxoglutaric acid NADPH NADP NADPH Glycolysis Mitochondrial Electron Transport Chain
MDH2 TET1 KDM2A MPC1 PDHA1 CS IDH3A IDH3B IDH3G OGDH SUCLG1 SUCLG2 FH PC SDHA SDHB SDHC SDHD ACO2 ACO2 gdhA GLS2 IDH1 IDH1 IDH2 ACO1 L2HGDH Pyr FADH2 Pyr CA CA CoA NAD Ac-CoA CO2 NADH H2O Oaa CoA I-cita NAD AKG CO2 NADH H+ NAD CoA Suc-CoA NADH H+ CO2 Pi GDP Succini CoA GTP Malate Fumarat H2O NAD NADH H+ ATP HCO3- ADP Pi Q FAD QH2 Aconiti H2O H2O NADPH Ammon H+ Glu H2O NADP Gln H2O NH3 I-cita NADP AKG NADPH NADPH 2HG NADP NADP AKG NADPH NADP NADPH ThiamPP Lipoamd FAD ThiamPP Lipoamd FAD Biotin Mn2+ FAD PW000146 PW000165 4Fe-4S 4Fe-4S 4Fe-4S Mg2+ Mg2+ Mitochondrial intermembrane space Mitochondrial matrix Mitochondrion Inner mitochondrial membrane Outer mitochondrial membrane Cytosol Epigenetic regulation: activation of enzymes that catalyze DNA demethylation. Oncogene activation Tumour-suppressor inactivation TCA cycle 2-Hydroxyglutarate mutIDH1 Methylcystosine dioxygenase TET1 Lysine-specific demethylase 2A Altered protein synthesis (oncogenic) Angiogenesis Cell proliferation
Mitochondria MDH2 TET1 KDM2A MPC1 PDHA1 CS IDH3A IDH3B IDH3G OGDH SUCLG1 SUCLG2 FH PC SDHA SDHB SDHC SDHD ACO2 ACO2 gdhA GLS2 IDH1 IDH1 IDH2 ACO1 L2HGDH Pyr FADH Pyr CA CA CoA NAD Ac-CoA CO2 NADH H2O Oaa CoA I-cita NAD AKG CO2 NADH H+ NAD CoA Suc-CoA NADH H+ CO2 Pi GDP Succini CoA GTP Malate Fumarat H2O NAD NADH H+ ATP HCO3 ADP Pi Coq FAD QH(2) Aconiti H2O H2O NADPH Ammon H+ Glu H2O NADP Gln H2O NH3 I-cita NADP AKG NADPH NADPH 2HG NADP NADP AKG NADPH NADP NADPH PW000146 PW000165