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Voltage- dependent anion-selective channel protein 1 Mitochondrial pyruvate carrier 1 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 CoA NAD Acetyl-CoA CO2 NADH H2O Oxalacetic acid Citric 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 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 Cytosol Mitochondrial matrix Mitochondrion Inner mitochondrial membrane Outer mitochondrial membrane TCA Cycle Intermembrane space Symptoms include hypotonia, lethargy, vomiting, tachypnea, and as the disease progresses As the disease progresses, it can cause developmental delays, cognitive disabilities, abnormal development of the face and head, and organ failure.
Mitochondria VDAC1 MPC1 PDHA1 CS IDH3A IDH3B IDH3G OGDH SUCLG1 SUCLG2 FH PC SDHA SDHB SDHC SDHD ACO2 ACO2 MDH2 Pyruvic acid Pyruvic acid Pyruvic acid Coenzyme A NAD Acetyl-CoA Carbon dioxide NADH Water Oxalacetic acid Citric acid Coenzyme A Isocitric acid NAD Oxoglutaric acid Carbon dioxide NADH Hydrogen Ion NAD Coenzyme A Succinyl-CoA NADH