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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
NH3
ADP
Pi
L-Glutamine
H2 O
NH3
Fructose 6-phosphate
Glucosamine 6-phosphate
L-Glutamic acid
Acetyl-CoA
N-Acetyl-D-Glucosamine
6-Phosphate
CoA
ADP
Chitin
ATP
ATP
Carbonic acid
H2 O
ADP
Pi
L-Glutamic acid
ATP
NH3
CO2
H2 O
ADP
Pi
PPi
L-Glutamic acid
Phosphoribosyl pyrophosphate
H2 O
ATP
Xanthylic acid
H2 O
AMP
PPi
L-Glutamic acid
Guanosine monophosphate
ATP
Nicotinic acid adenine
dinucleotide
H2 O
L-Glutamic acid
AMP
PPi
NAD
NAD
H2 O
NADH
L-Alanine
Oxoglutaric acid
Pyruvic acid
H2 O
NAD
NH3
NADH
L-Aspartic acid
Oxalacetic acid
ATP
AMP
PPi
ATP
L-Cysteine
γ-Glutamylcysteine
ADP
Pi
ATP
Glycine
Glutathione
ADP
Pi
Oxidized glutathione
NADPH
NADP
CO2
Oxoglutaric acid
Accumulation
Succinic acid semialdehyde
L-Glutamic acid
NAD
H2 O
Succinic acid
NADH
H+
ATP
AMP
PPi
Biotin
Magnesium
Zinc (II) ion
Magnesium
4Fe-4S
Pyridoxal
5'-phosphate
Pyridoxal
5'-phosphate
Magnesium
FAD
Pyridoxal
5'-phosphate
Pyridoxal
5'-phosphate
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
Intracellular Space
Unknown
Gls2
Gfpt1
Gnpnat1
Nagk
Cad
Cps1
Ppat
Gmps
Nadsyn1
Aldh4a1
Gpt
Glud1
Got2
Ears2
Gclc
Gclm
Gss
Gsr
Gad1
Abat
Aldh5a1
Qars
L-Glutamic acid
Carbamoyl
phosphate
5-
Phosphoribosylamine
Accumulation
γ-Aminobutyric
acid
1-Pyrroline-
5-carboxylic
acid
Adenosine
triphosphate
Ammonia
Adenosine
diphosphate
Phosphate
L-Glutamine
Water
Ammonia
Fructose
6-phosphate
Glucosamine
6-phosphate
L-Glutamic acid
Acetyl-CoA
N-Acetyl-D-
Glucosamine
6-Phosphate
Coenzyme A
Adenosine
diphosphate
ribose
Chitin
Adenosine
triphosphate
Adenosine
triphosphate
Carbonic acid
Water
Adenosine
diphosphate
Phosphoric acid
L-Glutamic acid
Adenosine
triphosphate
Ammonia
Carbon dioxide
Water
Adenosine
diphosphate
Phosphoric acid
Pyrophosphate
L-Glutamic acid
Phosphoribosyl
pyrophosphate
Water
Adenosine
triphosphate
Xanthylic acid
Water
Adenosine
monophosphate
Pyrophosphate
L-Glutamic acid
Guanosine
monophosphate
Adenosine
triphosphate
Nicotinic acid
adenine
dinucleotide
Water
L-Glutamic acid
Adenosine
monophosphate
Pyrophosphate
NAD
NAD
Water
NADH
L-Alanine
Oxoglutaric
acid
Pyruvic acid
Water
NAD
Ammonia
NADH
L-Aspartic acid
Oxalacetic acid
Adenosine
triphosphate
Adenosine
monophosphate
Pyrophosphate
Adenosine
triphosphate
L-Cysteine
γ-
Glutamylcysteine
Adenosine
diphosphate
ribose
Phosphate
Adenosine
triphosphate
Glycine
Glutathione
Adenosine
diphosphate
Phosphate
Oxidized
glutathione
NADPH
NADP
Carbon dioxide
Oxoglutaric
acid
Accumulation
Succinic acid
semialdehyde
L-Glutamic acid
NAD
Water
Succinic acid
NADH
Hydrogen Ion
Adenosine
triphosphate
Adenosine
monophosphate
Pyrophosphate
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
Gls2
Gfpt1
Gnpnat1
Nagk
Cad
Cps1
Ppat
Gmps
Nadsyn1
Aldh4a1
Gpt
Glud1
Got2
Ears2
Gclc
Gclm
Gss
Gsr
Gad1
Abat
Aldh5a1
Qars
Glu
CarbmP
5-PPRSA
Accumulation
GABA
1P5CA
ATP
NH3
ADP
Pi
Gln
H2 O
NH3
Fru6P
G6P
Glu
Ac-CoA
NAcG6P
CoA
ADP
Chitin
ATP
ATP
CarbncA
H2 O
ADP
Pi
Glu
ATP
NH3
CO2
H2 O
ADP
Pi
PPi
Glu
PRPP
H2 O
ATP
XntylcA
H2 O
AMP
PPi
Glu
GSMP
ATP
NctAADn
H2 O
Glu
AMP
PPi
NAD
NAD
H2 O
NADH
Ala
AKG
Pyr
H2 O
NAD
NH3
NADH
Asp
Oaa
ATP
AMP
PPi
ATP
Cys
γGluCys
ADP
Pi
ATP
Gly
GSH
ADP
Pi
GSSG
NADPH
NADP
CO2
AKG
Accumulation
SuccASA
Glu
NAD
H2 O
Succini
NADH
H+
ATP
AMP
PPi
Biotin
Mg2+
Zinc
Mg2+
4Fe-4S
Pyr-5'P
Pyr-5'P
Mg2+
FAD
Pyr-5'P
Pyr-5'P
TRNAGLU
LGTG
TRNAGLN
LGTG
PW000004
PW000160
PW000052
PW000043
PW000162
PW000010
PW000010
Intracellular Space
Gls2
Gfpt1
Gnpnat1
Nagk
Cad
Cps1
Ppat
Gmps
Nadsyn1
Aldh4a1
Gpt
Glud1
Got2
Ears2
Gclc
Gclm
Gss
Gsr
Gad1
Abat
Aldh5a1
Qars
Glu
CarbmP
5-PPRSA
Accumulation
GABA
1P5CA
ATP
NH3
ADP
Pi
Gln
H2O
NH3
Fru6P
G6P
Glu
Ac-CoA
NAcG6P
CoA
ADP-Rib
Chitin
ATP
ATP
CarbncA
H2O
ADP
PhosfrA
Glu
ATP
NH3
CO2
H2O
ADP
PhosfrA
Ppi
Glu
PRPP
H2O
ATP
XntylcA
H2O
AMP
Ppi
Glu
GSMP
ATP
NctAADn
H2O
Glu
AMP
Ppi
NAD
NAD
H2O
NADH
Ala
AKG
Pyr
H2O
NAD
NH3
NADH
Asp
Oaa
ATP
AMP
Ppi
ATP
Cys
γGluCys
ADP-Rib
Pi
ATP
Gly
GSH
ADP
Pi
GSSG
NADPH
NADP
CO2
AKG
Accumulation
SuccASA
Glu
NAD
H2O
Succini
NADH
H+
ATP
AMP
Ppi
TRNAGLU
LGTG
TRNAGLN
LGTG
PW000004
PW000160
PW000052
PW000043
PW000162
PW000010
PW000010