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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
GCSH
AQP8
SDS
ASRGL1
ASNS
HAL
CPS1
PIG59
GLUD1
GLS2
AMT
DLD
GLDC
Ser
His
Pyr
CarbmP
Gly
Glu
Glu
Asp
NH
3
NH
3
Asn
H
2
O
ATP
Gln
H
2
O
AMP
PP
i
Urocana
ATP
CO
2
H
2
O
ADP
P
i
ATP
ADP
P
i
Gln
H
2
O
NAD
AKG
NADH
H
2
O
Glu
THFA
8As6SA
Dhlip
5XM-THF
NAD
Lipoate
NADH
CO
2
Pyr-5'P
Biotin
Mg2+
FAD
PW000157
PW000043
PW000005
PW000162
PW000157
PW000003
PW000003
PW000002