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Mitochondria
SLC7A2
SLC25A2
Unknown
AASS
AASS
ALDH7A1
AADAT
ECHS1
HADH
ACAT1
PIPOX
GCDH
DHTKD1
Acetyl-CoA
L-Lysine
L-Lysine
L-Lysine
Allysine
Allysine
NADPH
Oxoglutaric
acid
Saccharopine
NADP
Water
Water
NAD
L-Glutamic acid
NADH
NAD
Water
Aminoadipic
acid
NADH
Oxoglutaric
acid
Oxoadipic acid
L-Glutamic acid
3-
Hydroxybutyryl-
CoA
Crotonoyl-CoA
Water
NAD
Acetoacetyl-CoA
NADH
Coenzyme A
L-Pipecolic
acid
Oxygen
(S)-2,3,4,5-
Tetrahydropiperidine-
2-carboxylate
Hydrogen
peroxide
Glutaryl-CoA
Carbon dioxide
Coenzyme A
Water
Citric Acid
Cycle
Biotin
Metabolism
SLC7A2
SLC25A2
AASS
AASS
ALDH7A1
AADAT
ECHS1
HADH
ACAT1
PIPOX
GCDH
DHTKD1
Ac-CoA
Lys
Lys
Lys
Alysine
Alysine
NADPH
AKG
Sacchar
NADP
H
2
O
H
2
O
NAD
Glu
NADH
NAD
H
2
O
AAD
NADH
AKG
Oxoadip
Glu
3HB-CoA
CrtylCA
H
2
O
NAD
ActaCoA
NADH
CoA
Pipecol
O
2
S-TH2C
H
2
O
2
GlutCoA
CO
2
CoA
H
2
O
Pyr-5'P
FAD
FAD
ThiamPP
Lipoamd
FAD
PW000005
PW000013
Inner Mitochondria Membrane
Outer Mitochondria Membrane
Mitochondrial Intermembrane
Space
Peroxisome
Mitochondria
Extracellular Space
Intracellular Space
Liver
Extracellular Space
Mitochondrial matrix