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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
CO
2
NADH
H
2
O
Oxalacetic acid
Citric acid
CoA
Isocitric acid
NAD
Oxoglutaric acid
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
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
Hydrogen Ion