Loader
Loading Pathway...
Voltage- dependent anion-selective channel protein 1 Mitochondrial pyruvate carrier 1 Solute carrier family 22 member 8 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 Malate dehydrogenase, mitochondrial Pyruvic acid Pyruvic acid Pyruvic acid Oxoglutaric acid Oxoglutaric acid CoA NAD Acetyl-CoA CO2 NADH H2O Oxalacetic acid Citric acid CoA Isocitric acid NAD CO2 NADH H+ NAD CoA Succinyl-CoA NADH H+ CO2 Pi GDP Succinic acid CoA GTP L-Malic acid Fumaric acid H2O ATP HCO3- ADP Pi Q FAD QH2 FADH2 cis-Aconitic acid H2O H2O NAD NADH H+ Thiamine pyrophosphate Lipoamide FAD Magnesium Thiamine pyrophosphate Lipoamide FAD Biotin Manganese 4Fe-4S 4Fe-4S Solute carrier family 22 member 8 Cytosol Mitochondrial matrix Mitochondrion Inner mitochondrial membrane Outer mitochondrial membrane TCA Cycle Intermembrane space Symptoms of the disorder include opisthotonus, ataxia, developmental delay, and seizures.
Mitochondria VDAC1 MPC1 SLC22A8 PDHA1 CS IDH3A IDH3B IDH3G OGDH SUCLG1 SUCLG2 FH PC SDHA SDHB SDHC SDHD ACO2 ACO2 MDH2 Pyruvic acid Pyruvic acid Pyruvic acid Oxoglutaric acid Oxoglutaric acid Coenzyme A NAD Acetyl-CoA Carbon dioxide NADH Water Oxalacetic acid 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 Succinic acid Coenzyme A Guanosine triphosphate L-Malic acid Fumaric acid Water Adenosine triphosphate Hydrogen carbonate Adenosine diphosphate Phosphate Coenzyme Q10 FAD QH(2) FADH cis-Aconitic acid Water Water NAD NADH Hydrogen Ion SLC22A8
VDAC1 MPC1 SLC22A8 PDHA1 CS IDH3A IDH3B IDH3G OGDH SUCLG1 SUCLG2 FH PC SDHA SDHB SDHC SDHD ACO2 ACO2 MDH2 Pyr Pyr Pyr AKG AKG CoA NAD Ac-CoA CO2 NADH H2O Oaa CA CoA I-cita NAD CO2 NADH H+ NAD CoA Suc-CoA NADH H+ CO2 Pi GDP Succini CoA GTP Malate Fumarat H2O ATP HCO3- ADP Pi Q FAD QH2 FADH2 Aconiti H2O H2O NAD NADH H+ ThiamPP Lipoamd FAD Mg2+ ThiamPP Lipoamd FAD Biotin Mn2+ 4Fe-4S 4Fe-4S SLC22A8 Cytosol Mitochondrial matrix Mitochondrion Inner mitochondrial membrane Outer mitochondrial membrane TCA Cycle Intermembrane space Symptoms of the disorder include opisthotonus, ataxia, developmental delay, and seizures.
Mitochondria VDAC1 MPC1 SLC22A8 PDHA1 CS IDH3A IDH3B IDH3G OGDH SUCLG1 SUCLG2 FH PC SDHA SDHB SDHC SDHD ACO2 ACO2 MDH2 Pyr Pyr Pyr AKG AKG CoA NAD Ac-CoA CO2 NADH H2O Oaa CA CoA I-cita NAD CO2 NADH H+ NAD CoA Suc-CoA NADH H+ CO2 Pi GDP Succini CoA GTP Malate Fumarat H2O ATP HCO3 ADP Pi Coq FAD QH(2) FADH Aconiti H2O H2O NAD NADH H+ SLC22A8