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Mitochondrial pyruvate carrier 1 Pyruvate dehydrogenase complex Citrate synthase, mitochondrial Isocitrate dehydrogenase [NAD] subunit alpha, mitochondrial Isocitrate dehydrogenase [NAD] subunit gamma 1, mitochondrial Isocitrate dehydrogenase [NAD] subunit gamma 1, mitochondrial Oxoglutarate dehydrogenase complex Succinyl-CoA ligase Fumarate hydratase, mitochondrial Pyruvate carboxylase, mitochondrial Succinate dehydrogenase Aconitate hydratase, mitochondrial Aconitate hydratase, mitochondrial Pyruvic acid Oxalacetic acid Accumulation Fumaric acid Succinic acid FADH2 Accumulation Oxoglutaric acid Acetyl-CoA Pyruvic acid 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 Thiamine pyrophosphate Lipoamide FAD Magnesium Thiamine pyrophosphate Lipoamide FAD Biotin Manganese 4Fe-4S 4Fe-4S 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 Inner mitochondrial membrane Mitochondrial intermembrane space Cytosol Mitochondrial matrix Mitochondrion Intracellular Space Inner mitochondrial membrane Outer mitochondrial membrane
Mitochondria Mpc1 Pdha1 Cs Idh3a Idh3g Idh3g Ogdh Suclg1 Suclg2 Fh Pc Sdha Sdhb Sdhc Sdhd Aco2 Aco2 Pyruvic acid Oxalacetic acid Accumulation Fumaric acid Succinic acid FADH Accumulation Oxoglutaric acid Acetyl-CoA Pyruvic acid 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 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
Mpc1 Pdha1 Cs Idh3a Idh3g Idh3g Ogdh Suclg1 Suclg2 Fh Pc Sdha Sdhb Sdhc Sdhd Aco2 Aco2 Pyr Oaa Accumulation Fumarat Succini FADH2 Accumulation AKG Ac-CoA Pyr 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 ThiamPP Lipoamd FAD Mg2+ ThiamPP Lipoamd FAD Biotin Mn2+ 4Fe-4S 4Fe-4S 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 Inner mitochondrial membrane Mitochondrial intermembrane space Cytosol Mitochondrial matrix Mitochondrion Intracellular Space Inner mitochondrial membrane Outer mitochondrial membrane
Mitochondria Mpc1 Pdha1 Cs Idh3a Idh3g Idh3g Ogdh Suclg1 Suclg2 Fh Pc Sdha Sdhb Sdhc Sdhd Aco2 Aco2 Pyr Oaa Accumulation Fumarat Succini FADH Accumulation AKG Ac-CoA Pyr 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 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