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Table of Primary Pathways
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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
Coenzyme A
Guanosine
triphosphate
L-Malic acid
Water
NAD
NADH
Hydrogen Ion
Adenosine
triphosphate
Hydrogen
carbonate
Adenosine
diphosphate
Phosphate
Coenzyme Q10
FAD
QH(2)
cis-Aconitic
acid
Water
Water
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
MPC1
PDHA1
CS
IDH3A
IDH3B
IDH3G
OGDH
SUCLG1
SUCLG2
FH
MDH2
PC
SDHA
SDHB
SDHC
SDHD
ACO2
ACO2
Pyr
Oaa
Accumulation
Fumarat
Succini
FADH
2
Accumulation
AKG
Ac-CoA
Pyr
CoA
NAD
CO
2
NADH
H
2
O
CA
CoA
I-cita
NAD
CO
2
NADH
H
+
NAD
CoA
Suc-CoA
NADH
H
+
CO
2
P
i
GDP
CoA