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Glycine
cleavage system
H protein,
mitochondrial
Aquaporin-8
L-serine
dehydratase/L-
threonine
deaminase
Isoaspartyl
peptidase/L-
asparaginase
Asparagine
synthetase
[glutamine-
hydrolyzing]
Histidine
ammonia-lyase
Carbamoyl-
phosphate
synthase
[ammonia],
mitochondrial
Glutamine
synthetase
Glutamate
dehydrogenase
1,
mitochondrial
Glutaminase
liver isoform,
mitochondrial
Aminomethyltransferase,
mitochondrial
Dihydrolipoyl
dehydrogenase,
mitochondrial
Glycine
dehydrogenase
[decarboxylating],
mitochondrial
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
Glycine and
Serine
Metabolism
Histidine
Metabolism
Citric Acid
Cycle
Urea Cycle
Glycine and
Serine
Metabolism
Glutamate
Metabolism
Glutamate
Metabolism
Aspartate
Metabolism
Pyridoxal
5'-phosphate
Glycine cleavage system
Mitochondrial Matrix
Extracellular Space
Cytoplasm
Mitochondria
Mitochondria
GCSH
AQP8
SDS
ASRGL1
ASNS
HAL
CPS1
PIG59
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