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Canalicular
multispecific
organic anion
transporter 2
Solute carrier
family 22
member 5
Mitochondrial
carnitine/acylcarnitine
carrier protein
CACL
Neutral amino
acid
transporter A
Calcium-binding
mitochondrial
carrier protein
Aralar1
Multidrug
resistance-
associated
protein 4
Mitochondrial
ornithine
transporter 1
Delta-1-
pyrroline-5-
carboxylate
synthase
Glutaminase
liver isoform,
mitochondrial
Ornithine
aminotransferase,
mitochondrial
Ornithine
carbamoyltransferase,
mitochondrial
Argininosuccinate
synthase
Argininosuccinate
lyase
Arginase,
mitochondrial
D-amino-acid
oxidase
Unknown
Argininic acid
L-Glutamine
L-Glutamine
L-Glutamine
Citrulline
Citrulline
L-Glutamine
Argininic acid
Ornithine
Ornithine
L-Glutamic acid
NADPH
H
+
1-Pyrroline-5-carboxylic acid
NADP
H
2
O
NH
3
Glutaric acid
Oxoglutaric acid
Carbamoyl phosphate
P
i
ATP
L-Aspartic acid
AMP
PP
i
Argininosuccinic acid
Fumaric acid
L-Arginine
H
2
O
Urea
NADP
H
2
O
2-Oxoarginine
NADPH
NH
3
NADPH
NADP
Calcium
Pyridoxal
5'-phosphate
FAD
Neurotoxicity
Glutamine is ingested
through food such as beef,
pork, poultry, dairy,
spinach, parsley, and
cabbage.
Intestine
Intestine Epithelial Cell
Blood vessel
Hepatocyte
Mitochondria
Mitochondrial Matrix
Cytosol
Low concentrations of
Arginase causes an
accumulation of Arginine,
and prevents the synthesis
of Urea and Ornithine.
Argininic acid is
synthesized by an unknown
hydrogenase
Argininic acid is a uremic
toxin which causes
neurological syndrome
consisting of a variable
degree of mental
retardation, epilepsy and
progressive spasticity.