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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
Malate
dehydrogenase,
mitochondrial
Pyruvate
carboxylase,
mitochondrial
Succinate
dehydrogenase
Aconitate
hydratase,
mitochondrial
Aconitate
hydratase,
mitochondrial
Pyruvic acid
Oxalacetic acid
Accumulation
Fumaric acid
Succinic acid
FADH
2
Accumulation
Oxoglutaric acid
Acetyl-CoA
Pyruvic acid
CoA
NAD
CO
2
NADH
H
2
O
Citric acid
CoA
Isocitric acid
NAD
CO
2
NADH
H
+
NAD
CoA
Succinyl-CoA
NADH
H
+
CO
2
P
i
GDP
CoA
GTP
L-Malic acid
H
2
O
NAD
NADH
H
+
ATP
HCO
3
-
ADP
P
i
Q
FAD
QH
2
cis-Aconitic acid
H
2
O
H
2
O
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
Inner mitochondrial membrane
Outer mitochondrial membrane
Mitochondria
MPC1
PDHA1
CS
IDH3A
IDH3B
IDH3G
OGDH
SUCLG1
SUCLG2
FH
MDH2
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