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Glycine
cleavage system
H protein,
mitochondrial
L-serine
dehydratase/L-
threonine
deaminase
Isoaspartyl
peptidase/L-
asparaginase
Asparagine
synthetase
[glutamine-
hydrolyzing]
Histidine
ammonia-lyase
Carbamoyl-
phosphate
synthase 1
Unknown
Glutamate
dehydrogenase
1,
mitochondrial
Glutaminase 2
Aminomethyltransferase,
mitochondrial
Dihydrolipoyl
dehydrogenase
Glycine
cleavage system
P protein
L-Serine
L-Histidine
Pyruvic acid
Carbamoyl
phosphate
Glycine
L-Glutamic acid
L-Glutamic acid
L-Aspartic acid
NH
3
NH
3
L-Asparagine
H
2
O
ATP
L-Glutamine
H
2
O
AMP
PP
i
Urocanic acid
ATP
CO
2
H
2
O
ADP
P
i
ATP
ADP
P
i
L-Glutamine
H
2
O
NAD
Oxoglutaric
acid
NADH
H
2
O
L-Glutamic acid
Tetrahydrofolic acid
8-[(Aminomethyl)sulfanyl]-6-
sulfanyloctanoic acid
Dihydrolipoate
5,10-Methylene-THF
NAD
(R)-lipoic acid
NADH
CO
2
Pyridoxal
5'-phosphate
Biotin
Magnesium
FAD
Pyridoxal
5'-phosphate
Glycine and
Serine
Metabolism
Histidine
Metabolism
Citric Acid
Cycle
Urea Cycle
Glycine and
Serine
Metabolism
Glutamate
Metabolism
Glutamate
Metabolism
Aspartate
Metabolism
Glycine cleavage system
Mitochondrial Matrix
Extracellular Space
Intracellular Space
Mitochondria
Mitochondria
GCSH
SDS
ASRGL1
ASNS
HAL
CPS1
Unknown
GLUD1
GLS2
AMT
DLD
GLDC
L-Serine
L-Histidine
Pyruvic acid
Carbamoyl
phosphate
Glycine
L-Glutamic acid
L-Glutamic acid
L-Aspartic acid
Ammonia
Ammonia
L-Asparagine
Water
Adenosine
triphosphate
L-Glutamine
Water
Adenosine
monophosphate
Pyrophosphate
Urocanic acid
Adenosine
triphosphate
Carbon dioxide
Water
Adenosine
diphosphate
Phosphoric acid
Adenosine
triphosphate
Adenosine
diphosphate
Phosphate
L-Glutamine
Water
NAD
Oxoglutaric
acid
NADH
Water
L-Glutamic acid
Tetrahydrofolic
acid
8-
[(Aminomethyl)sulfanyl]-
6-
sulfanyloctanoic
acid
Dihydrolipoate
5,10-
Methylene-THF
NAD
(R)-lipoic acid
NADH
Carbon dioxide
Glycine and
Serine
Metabolism
Histidine
Metabolism
Citric Acid
Cycle
Urea Cycle
Glycine and
Serine
Metabolism
Glutamate
Metabolism
Glutamate
Metabolism
Aspartate
Metabolism