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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
CO
2
NADH
H
2
O
Oxalacetic acid
Citric acid
CoA
Isocitric acid
NAD
CO
2
NADH
H
+
NAD
CoA
Succinyl-CoA
NADH
H
+
CO
2
P
i
GDP
Succinic acid
CoA
GTP
L-Malic acid
Fumaric acid
H
2
O
ATP
HCO
3
-
ADP
P
i
Q
FAD
QH
2
FADH
2
cis-Aconitic acid
H
2
O
H
2
O
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.