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Mitochondrial pyruvate carrier 1 Hexokinase-2 Hexokinase-2 Aldose 1-epimerase Hexokinase-2 Glucose-6- phosphate isomerase Glucose-6- phosphate isomerase 6- phosphofructokinase, muscle type Fructose- bisphosphate aldolase A Triosephosphate isomerase Glucose-6- phosphatase Glyceraldehyde- 3-phosphate dehydrogenase Bisphosphoglycerate mutase Phosphoglycerate mutase 1 Bisphosphoglycerate mutase Phosphoglycerate mutase 2 Alpha-enolase Pyruvate dehydrogenase complex Citrate synthase, 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 Bifunctional protein BirA Acetyl-coenzyme A carboxylase carboxyl transferase subunit alpha Acetyl-coenzyme A carboxylase carboxyl transferase subunit beta Biotin carboxylase Biotin carboxyl carrier protein of acetyl-CoA carboxylase Acetyl-coenzyme A carboxylase carboxyl transferase subunit alpha Acetyl-coenzyme A carboxylase carboxyl transferase subunit beta Adenylate cyclase type 2 Guanine nucleotide- binding protein G(s) subunit alpha isoforms short Adenylate cyclase type 2 Guanine nucleotide- binding protein G(s) subunit alpha isoforms short 5- hydroxytryptamine receptor 4 5- hydroxytryptamine receptor 4 Guanine nucleotide- binding protein G(T) subunit gamma-T1 Gβ Pyruvic acid Oxalacetic acid Fumaric acid Succinic acid FADH2 Oxoglutaric acid Acetyl-CoA Pyruvic acid D-Glucose ATP Glucose 6-phosphate ADP α-D-Glucose ATP ADP β-D-Glucose ATP ADP β-D-Glucose 6-phosphate Fructose 6-phosphate ATP Fructose 1,6-bisphosphate ADP D-Glyceraldehyde 3-phosphate Dihydroxyacetone phosphate Glucose 1-phosphate H2O Pi NAD Pi Glyceric acid 1,3-biphosphate NADH 2,3- Diphosphoglyceric acid ADP 3-Phosphoglyceric acid ATP H2O Pi H2O Pi 2-Phospho-D- glyceric acid Phosphoenolpyruvic acid H2O CoA NAD CO2 NADH H2O Citric acid CoA Isocitric acid NAD CO2 NADH H+ NAD CoA Succinyl-CoA NADH H+ CO2 Pi GDP CoA GTP L-Malic acid H2O NAD NADH H+ ATP HCO3- ADP Pi Q FAD QH2 cis-Aconitic acid H2O H2O ATP Biotin H+ AMP PPi ATP HCO3- H+ ADP Pi H+ Pi ADP Malonyl-CoA H2O Acetyl-CoA ATP GTP GDP 1-Methylhistidine Magnesium Magnesium Thiamine pyrophosphate Lipoamide FAD Magnesium Thiamine pyrophosphate Lipoamide FAD Biotin Manganese 4Fe-4S 4Fe-4S Magnesium Guanosine triphosphate Magnesium Biotin-Carboxyl Carrying Protein Biotinylated [BCCP monomer] a biotinylated [BCCP dimer] a biotinylated [BCCP dimer] carboxylated- biotinylated [BCCP dimer] fused chorismate mutase T/prephenate dehydrogenase 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 Isocitrate dehydrogenase [NAD] subunit alpha, mitochondrial Inner mitochondrial membrane Mitochondrial intermembrane space Cytosol Mitochondrial matrix Mitochondrion Intracellular Space Inner mitochondrial membrane Outer mitochondrial membrane
Mitochondria MPC1 HK2 HK2 GALM HK2 GPI GPI PFKM ALDOA TPI1 G6PC GAPDH BPGM PGAM1 BPGM PGAM2 ENO1 PDHA1 CS IDH3B IDH3G OGDH SUCLG1 SUCLG2 FH PC SDHA SDHB SDHC SDHD ACO2 ACO2 birA accA accD accC accB accA accD ADCY2 GNAS ADCY2 GNAS HTR4 HTR4 GNGT1 GNB1 Pyruvic acid Oxalacetic acid Fumaric acid Succinic acid FADH Oxoglutaric acid Acetyl-CoA Pyruvic acid D-Glucose Adenosine triphosphate Glucose 6-phosphate Adenosine diphosphate α-D-Glucose Adenosine triphosphate Adenosine diphosphate β-D-Glucose Adenosine triphosphate Adenosine diphosphate β-D-Glucose 6-phosphate Fructose 6-phosphate Adenosine triphosphate Fructose 1,6-bisphosphate Adenosine diphosphate D-Glyceraldehyde 3-phosphate Dihydroxyacetone phosphate Glucose 1-phosphate Water Phosphate NAD Phosphoric acid Glyceric acid 1,3-biphosphate NADH 2,3- Diphosphoglyceric acid Adenosine diphosphate 3-Phosphoglyceric acid Adenosine triphosphate Water Phosphate Water Phosphate 2-Phospho-D- glyceric acid Phosphoenolpyruvic acid Water Coenzyme A NAD Carbon dioxide NADH Water Citric acid Coenzyme A Isocitric acid NAD Carbon dioxide NADH Hydrogen Ion NAD Coenzyme A Succinyl-CoA NADH Hydrogen Ion Carbon dioxide Phosphate Guanosine diphosphate Coenzyme A Guanosine triphosphate L-Malic acid Water NAD NADH Hydrogen Ion Adenosine triphosphate Hydrogen carbonate Adenosine diphosphate Phosphate Coenzyme Q10 FAD QH(2) cis-Aconitic acid Water Water Adenosine triphosphate Biotin Hydrogen Ion Adenosine monophosphate Pyrophosphate Adenosine triphosphate Hydrogen carbonate Hydrogen Ion Adenosine diphosphate Phosphate Hydrogen Ion Phosphate Adenosine diphosphate Malonyl-CoA Water Acetyl-CoA Adenosine triphosphate Guanosine triphosphate Guanosine diphosphate 1-Methylhistidine Biotin-Carboxyl Carrying Protein Biotinylated [BCCP monomer] a biotinylated [BCCP dimer] a biotinylated [BCCP dimer] carboxylated- biotinylated [BCCP dimer] fused chorismate mutase T/prephenate dehydrogenase 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 IDH3A
MPC1 HK2 HK2 GALM HK2 GPI GPI PFKM ALDOA TPI1 G6PC GAPDH BPGM PGAM1 BPGM PGAM2 ENO1 PDHA1 CS IDH3B IDH3G OGDH SUCLG1 SUCLG2 FH PC SDHA SDHB SDHC SDHD ACO2 ACO2 birA accA accD accC accB accA accD ADCY2 GNAS ADCY2 GNAS HTR4 HTR4 GNGT1 GNB1 Pyr Oaa Fumarat Succini FADH2 AKG Ac-CoA Pyr D-Glc ATP Gluc-6P ADP a-D-Glc ATP ADP Glu ATP ADP BDGlu6P Fru6P ATP Fru1,6P ADP Glyc3P Dhapp Gluc1P H2O Pi NAD Pi GlyABp NADH 23-DpGA ADP G3P ATP H2O Pi H2O Pi 2PDG Pep H2O CoA NAD CO2 NADH H2O CA CoA I-cita NAD CO2 NADH H+ NAD CoA Suc-CoA NADH H+ CO2 Pi GDP CoA GTP Malate H2O NAD NADH H+ ATP HCO3- ADP Pi Q FAD QH2 Aconiti H2O H2O ATP Biotin H+ AMP PPi ATP HCO3- H+ ADP Pi H+ Pi ADP MalyCoA H2O Ac-CoA ATP GTP GDP 1-Mhis Mg2+ Mg2+ ThiamPP Lipoamd FAD Mg2+ ThiamPP Lipoamd FAD Biotin Mn2+ 4Fe-4S 4Fe-4S Mg2+ GTP Mg2+ BCCP BBM ABBD ABBD CBBD FCMTD PW000152 PW000146 PW000054 PW000030 PW000152 PW000001 PW000002 PW000054 PW000002 PW000142 PW000010 PW000162 PW000149 PW000165 PW000030 PW000003 PW000001 PW000152 PW000046 PW000051 PW000163 PW000054 PW000023 PW000028 PW000029 PW000155 PW000149 IDH3A Inner mitochondrial membrane Mitochondrial intermembrane space Cytosol Mitochondrial matrix Mitochondrion Intracellular Space Inner mitochondrial membrane Outer mitochondrial membrane
Mitochondria MPC1 HK2 HK2 GALM HK2 GPI GPI PFKM ALDOA TPI1 G6PC GAPDH BPGM PGAM1 BPGM PGAM2 ENO1 PDHA1 CS IDH3B IDH3G OGDH SUCLG1 SUCLG2 FH PC SDHA SDHB SDHC SDHD ACO2 ACO2 birA accA accD accC accB accA accD ADCY2 GNAS ADCY2 GNAS HTR4 HTR4 GNGT1 GNB1 Pyr Oaa Fumarat Succini FADH AKG Ac-CoA Pyr D-Glc ATP Gluc-6P ADP a-D-Glc ATP ADP Glu ATP ADP BDGlu6P Fru6P ATP Fru1,6P ADP Glyc3P Dhapp Gluc1P H2O Pi NAD PhosfrA GlyABp NADH 23-DpGA ADP G3P ATP H2O Pi H2O Pi 2PDG Pep H2O CoA NAD CO2 NADH H2O CA CoA I-cita NAD CO2 NADH H+ NAD CoA Suc-CoA NADH H+ CO2 Pi GDP CoA GTP Malate H2O NAD NADH H+ ATP HCO3 ADP Pi Coq FAD QH(2) Aconiti H2O H2O ATP Biotin H+ AMP Ppi ATP HCO3 H+ ADP Pi H+ Pi ADP MalyCoA H2O Ac-CoA ATP GTP GDP 1-Mhis BCCP BBM ABBD ABBD CBBD FCMTD PW000152 PW000146 PW000054 PW000030 PW000152 PW000001 PW000002 PW000054 PW000002 PW000142 PW000010 PW000162 PW000149 PW000165 PW000030 PW000003 PW000001 PW000152 PW000046 PW000051 PW000163 PW000054 PW000023 PW000028 PW000029 PW000155 PW000149 IDH3A