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Glutamine
synthetase
Glutaminase
liver isoform,
mitochondrial
Glutamine--
fructose-6-
phosphate
aminotransferase
[isomerizing] 1
Glucosamine
6-phosphate N-
acetyltransferase
N-acetyl-D-
glucosamine
kinase
CAD protein
Carbamoyl-
phosphate
synthase
[ammonia],
mitochondrial
Amidophosphoribosyltransferase
GMP synthase
[glutamine-
hydrolyzing]
Glutamine-
dependent
NAD(+)
synthetase
Delta-1-
pyrroline-5-
carboxylate
dehydrogenase,
mitochondrial
Alanine
aminotransferase
1
Glutamate
dehydrogenase
1,
mitochondrial
Aspartate
aminotransferase,
mitochondrial
Probable
glutamate--tRNA
ligase,
mitochondrial
Glutamate--
cysteine ligase
Glutathione
synthetase
Glutathione
reductase,
mitochondrial
Glutamate
decarboxylase 1
4-aminobutyrate
aminotransferase,
mitochondrial
Succinate-
semialdehyde
dehydrogenase,
mitochondrial
Glutamine--tRNA
ligase
L-Glutamic acid
Carbamoyl phosphate
5-Phosphoribosylamine
Accumulation
γ-Aminobutyric acid
1-Pyrroline-5-carboxylic acid
ATP
NH
3
ADP
P
i
L-Glutamine
H
2
O
NH
3
Fructose 6-phosphate
Glucosamine 6-phosphate
L-Glutamic acid
Acetyl-CoA
N-Acetyl-D-Glucosamine
6-Phosphate
CoA
ADP
Chitin
ATP
ATP
Carbonic acid
H
2
O
ADP
P
i
L-Glutamic acid
ATP
NH
3
CO
2
H
2
O
ADP
P
i
PP
i
L-Glutamic acid
Phosphoribosyl pyrophosphate
H
2
O
ATP
Xanthylic acid
H
2
O
AMP
PP
i
L-Glutamic acid
Guanosine monophosphate
ATP
Nicotinic acid adenine
dinucleotide
H
2
O
L-Glutamic acid
AMP
PP
i
NAD
NAD
H
2
O
NADH
L-Alanine
Oxoglutaric acid
Pyruvic acid
H
2
O
NAD
NH
3
NADH
L-Aspartic acid
Oxalacetic acid
ATP
AMP
PP
i
ATP
L-Cysteine
γ-Glutamylcysteine
ADP
P
i
ATP
Glycine
Glutathione
ADP
P
i
Oxidized glutathione
NADPH
NADP
CO
2
Oxoglutaric acid
Accumulation
Succinic acid semialdehyde
L-Glutamic acid
NAD
H
2
O
Succinic acid
NADH
H
+
ATP
AMP
PP
i
Biotin
Magnesium
Zinc (II) ion
Magnesium
4Fe-4S
tRNA(Glu)
L- Glutamyl-
tRNA(Glu)
tRNA(Gln)
L-
Glutaminyl-
tRNA(Gln)
Glutathione
Metabolism
Pyrimidine
Metabolism
Purine
Metabolism
Histidine
Metabolism
Urea Cycle
Arginine and
Proline
Metabolism
Arginine and
Proline
Metabolism
Pyridoxal
5'-phosphate
Pyridoxal
5'-phosphate
Magnesium
FAD
Pyridoxal
5'-phosphate