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Mitochondrial
ornithine
transporter 1
Mitochondrial
ornithine
transporter 1
Argininosuccinate
synthase
Argininosuccinate
lyase
Carbamoyl-
phosphate
synthase
[ammonia],
mitochondrial
Glutamate
dehydrogenase
1,
mitochondrial
Ornithine
aminotransferase,
mitochondrial
Delta-1-
pyrroline-5-
carboxylate
dehydrogenase,
mitochondrial
Delta-1-
pyrroline-5-
carboxylate
dehydrogenase,
mitochondrial
Proline
dehydrogenase
1,
mitochondrial
Pyrroline-5-
carboxylate
reductase 2
Prolyl
4-hydroxylase
subunit alpha-3
Proline
dehydrogenase
1,
mitochondrial
Pyrroline-5-
carboxylate
reductase 2
Probable
arginine--tRNA
ligase,
mitochondrial
Glycine
amidinotransferase,
mitochondrial
Guanidinoacetate
N-
methyltransferase
Creatine kinase
B-type
Arginase-1
Ornithine
carbamoyltransferase,
mitochondrial
D-amino-acid
oxidase
D-amino-acid
oxidase
Delta-1-
pyrroline-5-
carboxylate
dehydrogenase,
mitochondrial
Proline
dehydrogenase
1,
mitochondrial
Delta-1-
pyrroline-5-
carboxylate
dehydrogenase,
mitochondrial
Aspartate
aminotransferase,
cytoplasmic
Nitric oxide
synthase, brain
Bifunctional
glutamate/proline-
-tRNA ligase
Fumaric acid
L-Aspartic acid
Accumulation
Ornithine
L-Arginine
Citrulline
Citrulline
Accumulation
Ornithine
ATP
AMP
PP
i
Argininosuccinic acid
ATP
NH
3
CO
2
H
2
O
ADP
Phosphoric acid
Carbamoyl phosphate
L-Glutamic acid
H
2
O
NAD
Oxoglutaric acid
NADH
Oxoglutaric acid
L-Glutamic γ-semialdehyde
NAD
H
2
O
NADH
H
+
1-Pyrroline-5-carboxylic acid
NAD
H
2
O
NADH
L-Proline
NAD
NADH
Oxoglutaric acid
O
2
4-Hydroxyproline
Succinic acid
CO
2
Pyrroline hydroxycarboxylic
acid
NAD
NADH
ATP
AMP
PP
i
Glycine
Accumulation
Ornithine
Guanidoacetic acid
S-Adenosylmethionine
S-Adenosylhomocysteine
Creatine
ATP
ADP
Phosphocreatine
H
2
O
Urea
P
i
D-Proline
H
2
O
O
2
1-Pyrroline-2-carboxylic acid
NH
3
H
2
O
2
H
2
O
O
2
1-Pyrroline-4-hydroxy-2-
carboxylate
NH
3
H
2
O
2
NAD
H
2
O
4-Hydroxy-L-glutamic acid
NADPH
L-4-Hydroxyglutamate
semialdehyde
NAD
H
2
O
NADPH
Oxalacetic acid
4-Hydroxy-2-oxoglutaric acid
L-Aspartic acid
NADPH
O
2
N(omega)-Hydroxyarginine
NO
NADP
H
2
O
ATP
AMP
PP
i
FAD
Manganese
Pyridoxal
5'-phosphate
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
Pyridoxal
5'-phosphate
FAD
Heme
Flavin
Mononucleotide
Tetrahydrobiopterin
FAD
FAD
FAD
FAD
Urea Cycle
Cytosol
Extracellular Space
Mitochondria
Lysosome
Mitochondria
Mitochondrial Matrix