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Mitochondria
SLC25A29
ASS1
ASL
CPS1
GLUD1
OAT
ALDH4A1
ALDH4A1
PRODH
PYCR2
P4HA3
PRODH
PYCR2
RARS2
GATM
GAMT
CKB
ARG1
OTC
DDO
DDO
ALDH4A1
PRODH
ALDH4A1
GOT1
NOS3
EPRS
Fumaric acid
L-Aspartic acid
Ornithine
L-Arginine
Ornithine
Citrulline
Citrulline
Adenosine
triphosphate
Adenosine
monophosphate
Pyrophosphate
Argininosuccinic
acid
Adenosine
triphosphate
Ammonia
Carbon dioxide
Water
Adenosine
diphosphate
Phosphoric acid
Carbamoyl
phosphate
L-Glutamic acid
Water
NAD
Oxoglutaric
acid
NADH
Oxoglutaric
acid
L-Glutamic
γ-semialdehyde
NAD
Water
NADH
Hydrogen Ion
1-Pyrroline-
5-carboxylic
acid
NAD
Water
NADH
L-Proline
NAD
NADH
Oxoglutaric
acid
Oxygen
4-
Hydroxyproline
Succinic acid
Carbon dioxide
Pyrroline
hydroxycarboxylic
acid
NAD
NADH
Adenosine
triphosphate
Adenosine
monophosphate
Pyrophosphate
Glycine
Ornithine
Guanidoacetic
acid
S-
Adenosylmethionine
S-
Adenosylhomocysteine
Creatine
Adenosine
triphosphate
Adenosine
diphosphate
Phosphocreatine
Water
Urea
Phosphate
D-Proline
Water
Oxygen
1-Pyrroline-
2-carboxylic
acid
Ammonia
Hydrogen
peroxide
Water
Oxygen
1-Pyrroline-
4-hydroxy-2-
carboxylate
Ammonia
Hydrogen
peroxide
NAD
Water
4-Hydroxy-L-
glutamic acid
NADPH
L-4-
Hydroxyglutamate
semialdehyde
NAD
Water
NADPH
Oxalacetic acid
4-Hydroxy-2-
oxoglutaric
acid
L-Aspartic acid
NADPH
Oxygen
N(omega)-
Hydroxyarginine
Nitric oxide
NADP
Water
Adenosine
triphosphate
Adenosine
monophosphate
Pyrophosphate
tRNA(Arg)
L-Arginyl-
tRNA(Arg)
tRNA(Pro)
L-Prolyl-
tRNA(Pro)
Citric Acid
Cycle
Alanine
Metabolism
D-Arginine and
D-Ornithine
Metabolism
D-Arginine and
D-Ornithine
Metabolism