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Neutral amino
acid
transporter
B(0)
Neutral amino
acid
transporter A
Mitochondrial
ornithine
transporter 1
Mitochondrial
ornithine
transporter 1
Calcium-binding
mitochondrial
carrier protein
Aralar1
Alanine
aminotransferase
1
Glutaminase
liver isoform,
mitochondrial
Glutamate
dehydrogenase
1,
mitochondrial
Carbamoyl-
phosphate
synthase
[ammonia],
mitochondrial
Aspartate
aminotransferase,
mitochondrial
Ornithine
carbamoyltransferase,
mitochondrial
Arginase-1
Argininosuccinate
lyase
Argininosuccinate
synthase
L-Glutamine
L-Alanine
Oxoglutaric acid
L-Glutamic acid
Oxalacetic acid
NH
3
L-Aspartic acid
Fumaric acid
Accumulation
L-Arginine
Ornithine
L-Glutamine
L-Alanine
Ornithine
Citrulline
Citrulline
Ornithine
Ornithine
Citrulline
Citrulline
L-Aspartic acid
L-Glutamic acid
L-Glutamic acid
Pyruvic acid
H
2
O
H
2
O
NAD
Oxoglutaric acid
NADH
ATP
CO
2
H
2
O
ADP
P
i
Carbamoyl phosphate
P
i
H
2
O
Urea
Accumulation
Argininosuccinic acid
ATP
AMP
PP
i
Calcium
Pyridoxal
5'-phosphate
Pyridoxal
5'-phosphate
Manganese
Ammonia
Recycling
Pyrimidine
Metabolism
Glutamate
Metabolism
Alanine
Metabolism
Glucose-Alanine
Cycle
Selenoamino
Acid Metabolism
Malate-
Aspartate
Shuttle
Glutamate
Metabolism
Alanine
Metabolism
Glucose-Alanine
Cycle
Gluconeogenesis