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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
Mitochondrial
ornithine
transporter 1
Low affinity
cationic amino
acid
transporter 2
Delta-1-
pyrroline-5-
carboxylate
synthase
Glutaminase
liver isoform,
mitochondrial
Ornithine
aminotransferase,
mitochondrial
Ornithine
carbamoyltransferase,
mitochondrial
Argininosuccinate
synthase
Argininosuccinate
lyase
Arginase,
mitochondrial
Histone
acetyltransferase
type B
catalytic
subunit
N-a-Acetyl-L-arginine
L-Glutamine
L-Glutamine
L-Glutamine
Citrulline
Citrulline
L-Glutamine
Ornithine
Ornithine
N-a-Acetyl-L-arginine
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
Acetyl-CoA
CoA
Calcium
Pyridoxal
5'-phosphate
Neurological
Deficits
Kidney damage
Cardiovascular
Disease
Glutamine is ingested
through food
Intestine
Intestine Epithelial Cell
Blood vessel
Liver Hepatic Cell
Mitochondria
Mitochondrial Matrix
Cytosol
N-a-Acetyl-L-arginine is an
uremic toxin. Like other
argininic uremic toxins high
levels of guanidino in
cerebrospinal fluid.