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Solute carrier
family 23
member 1
Bis(5'-
nucleosyl)-
tetraphosphatase
[asymmetrical]
Nucleoside
diphosphate
kinase 6
Ectonucleoside
triphosphate
diphosphohydrolase
5
Ectonucleoside
triphosphate
diphosphohydrolase
8
Guanylate
kinase
Nucleoside
diphosphate
kinase 6
Guanylate
kinase
Cytosolic
purine
5'-nucleotidase
Deoxyguanosine
kinase
Adenine
phosphoribosyltransferase
Cytosolic
purine
5'-nucleotidase
Purine
nucleoside
phosphorylase
Hypoxanthine-
guanine
phosphoribosyltransferase
Purine
nucleoside
phosphorylase
Guanine
deaminase
ADP-sugar
pyrophosphatase
Ribose-
phosphate
pyrophosphokinase
3
Amidophosphoribosyltransferase
Trifunctional
purine
biosynthetic
protein
adenosine-3
Trifunctional
purine
biosynthetic
protein
adenosine-3
Phosphoribosylformylglycinamidine
synthase
Multifunctional
protein ADE2
Multifunctional
protein ADE2
Adenylosuccinate
lyase
Bifunctional
purine
biosynthesis
protein PURH
Bifunctional
purine
biosynthesis
protein PURH
Inosine
triphosphate
pyrophosphatase
GMP synthase
[glutamine-
hydrolyzing]
Inosine-5'-
monophosphate
dehydrogenase 1
GMP reductase 1
Cytosolic
purine
5'-nucleotidase
Purine
nucleoside
phosphorylase
Cytosolic
purine
5'-nucleotidase
Hypoxanthine-
guanine
phosphoribosyltransferase
Purine
nucleoside
phosphorylase
Hypoxanthine-
guanine
phosphoribosyltransferase
Xanthine
dehydrogenase/oxidase
Xanthine
dehydrogenase/oxidase
Purine
nucleoside
phosphorylase
Adenylosuccinate
synthetase
isozyme 2
AMP deaminase 1
Adenylosuccinate
lyase
Adenosine
deaminase
Purine
nucleoside
phosphorylase
Purine
nucleoside
phosphorylase
Adenosine
deaminase
Adenine
phosphoribosyltransferase
Ectonucleoside
triphosphate
diphosphohydrolase
5
Adenylate
kinase
isoenzyme 1
ATPase family
AAA domain-
containing
protein 1
cAMP-specific
3',5'-cyclic
phosphodiesterase
4D
Adenylate
cyclase type 2
Adenylate
kinase
isoenzyme 1
Cytosolic
purine
5'-nucleotidase
Nucleoside
diphosphate
kinase 6
Guanylate
cyclase
cAMP and
cAMP-inhibited
cGMP
3',5'-cyclic
phosphodiesterase
10A
Ectonucleoside
triphosphate
diphosphohydrolase
8
Inosine
triphosphate
pyrophosphatase
Nucleoside
diphosphate
kinase 6
Ectonucleoside
triphosphate
diphosphohydrolase
8
Adenine
phosphoribosyltransferase
Trifunctional
purine
biosynthetic
protein
adenosine-3
Thioredoxin
Thioredoxin
Ribonucleoside-
diphosphate
reductase large
subunit
Ribonucleoside-
diphosphate
reductase
subunit M2
Ectonucleoside
triphosphate
diphosphohydrolase
5
Thioredoxin
Thioredoxin
Ribonucleoside-
diphosphate
reductase large
subunit
Ribonucleoside-
diphosphate
reductase
subunit M2
Phosphoribosyl pyrophosphate
Xanthine
Diguanosine tetraphosphate
H2 O
Guanosine triphosphate
Guanosine monophosphate
ATP
Guanosine diphosphate
ADP
H2 O
Guanosine monophosphate
Pi
H2 O
Pi
ATP
ADP
ATP
dGDP
ADP
dGTP
ATP
2'-Deoxyguanosine
5'-monophosphate
ADP
H2 O
Deoxyguanosine
Pi
ATP
ADP
PPi
Guanine
Phosphoribosyl pyrophosphate
H2 O
Guanosine
Pi
Pi
Ribose
1-phosphate
PPi
Phosphoribosyl pyrophosphate
Pi
Deoxyribose
1-phosphate
H2 O
NH3
Adenosine diphosphate ribose
H2 O
AMP
D-Ribose 5-phosphate
ATP
AMP
5-Phosphoribosylamine
PPi
L-Glutamic acid
L-Glutamine
H2 O
ATP
Glycine
ADP
Pi
Glycineamideribotide
10-Formyltetrahydrofolate
Tetrahydrofolic acid
5'-Phosphoribosyl-N-
formylglycinamide
ATP
H2 O
ADP
Pi
Phosphoribosylformylglycineamidine
5-amino-1-(5-phospho-D-
ribosyl)imidazole-4-
carboxylate
5-Aminoimidazole
ribonucleotide
CO2
ATP
L-Aspartic acid
ADP
Pi
SAICAR
Fumaric acid
AICAR
10-Formyltetrahydrofolate
Tetrahydrofolic acid
Phosphoribosyl
formamidocarboxamide
Inosinic acid
H2 O
H2 O
PPi
ATP
Xanthylic acid
NH3
AMP
PPi
NAD
H2 O
NADH
NH3
NADP
NADPH
H2 O
Xanthosine
Pi
Pi
Ribose 1-phosphate
H2 O
Inosine
Pi
PPi
Phosphoribosyl pyrophosphate
Pi
Ribose 1-phosphate
PPi
O2
H2 O
H2 O2
NAD
H2 O
Uric acid
NADH
Deoxyinosine
Pi
Deoxyribose 1-phosphate
GTP
L-Aspartic acid
GDP
Pi
Adenylsuccinic acid
Adenosine monophosphate
H2 O
NH3
Fumaric acid
Adenosine
H2 O
NH3
Pi
Adenine
Ribose 1-phosphate
Deoxyadenosine
Pi
Deoxyribose 1-phosphate
H2 O
NH3
PPi
Phosphoribosyl pyrophosphate
Adenosine diphosphate
H2 O
Pi
Adenosine triphosphate
H2 O
Pi
cAMP
H2 O
PPi
ATP
Deoxyadenosine monophosphate
ADP
dADP
H2 O
Pi
ATP
ADP
Deoxyadenosine triphosphate
Guanosine 2',3'-cyclic
phosphate
PPi
H2 O
IDP
H2 O
Pi
Inosine triphosphate
H2 O
PPi
ATP
ADP
H2 O
Pi
PPi
5-Aminoimidazole-4-carboxamide
Phosphoribosyl pyrophosphate
ATP
ADP
Pi
H2 O
Pi
H2 O
H2 O
Calcium
Magnesium
Zinc (II) ion
Magnesium
Magnesium
Magnesium
Potassium
FAD
FAD
Magnesium
Zinc (II) ion
Zinc (II) ion
Zinc (II) ion
Calcium
Magnesium
Magnesium
Magnesium
Magnesium
Heme
Magnesium
Calcium
Disulfide
Calcium
Magnesium
Disulfide
Xanthine
Hypoxanthine
Hypoxanthine
Pentose
Phosphate
Pathway
Magnesium
Magnesium
Magnesium
Magnesium
Magnesium
Magnesium
Magnesium
Magnesium
Zinc (II) ion
Zinc (II) ion
Magnesium
Calcium
Magnesium
Fe3+
Fe3+
Peroxisome
Magnesium
Calcium
Endoplasmic Reticulum
Endoplasmic Reticulum
Peroxisome
Intracellular Space
Extracellular Space
Mitochondria
Cytosol
Mitochondria
Symptoms include arthralgia,
hematuria, mental
retardation, stomatisis, and
urolithiasis.
Kidney
SLC23A1
NUDT2
NME6
ENTPD5
ENTPD8
GUK1
NME6
GUK1
NT5C2
DGUOK
APRT
NT5C2
PNP
HPRT1
PNP
GDA
NUDT5
PRPS1L1
PPAT
GART
GART
PFAS
PAICS
PAICS
ADSL
ATIC
ATIC
ITPA
GMPS
IMPDH1
GMPR
NT5C2
PNP
NT5C2
HPRT1
PNP
HPRT1
XDH
XDH
PNP
ADSS
AMPD1
ADSL
ADA
PNP
PNP
ADA
APRT
ENTPD5
AK1
ATAD1
PDE4D
ADCY2
AK1
NT5C2
NME6
GUCY1A2
GUCY1B3
PDE10A
ENTPD8
ITPA
NME6
ENTPD8
APRT
GART
TXN
TXN
RRM1
RRM2
ENTPD5
TXN
TXN
RRM1
RRM2
Phosphoribosyl
pyrophosphate
Xanthine
Diguanosine
tetraphosphate
Water
Guanosine
triphosphate
Guanosine
monophosphate
Adenosine
triphosphate
Guanosine
diphosphate
Adenosine
diphosphate
Water
Guanosine
monophosphate
Phosphate
Water
Phosphate
Adenosine
triphosphate
Adenosine
diphosphate
Adenosine
triphosphate
dGDP
Adenosine
diphosphate
dGTP
Adenosine
triphosphate
2'-
Deoxyguanosine
5'-
monophosphate
Adenosine
diphosphate
Water
Deoxyguanosine
Phosphate
Adenosine
triphosphate
Adenosine
diphosphate
Pyrophosphate
Guanine
Phosphoribosyl
pyrophosphate
Water
Guanosine
Phosphoric acid
Phosphate
Ribose
1-phosphate
Pyrophosphate
Phosphoribosyl
pyrophosphate
Phosphate
Deoxyribose
1-phosphate
Water
Ammonia
Adenosine
diphosphate
ribose
Water
Adenosine
monophosphate
D-Ribose
5-phosphate
Adenosine
triphosphate
Adenosine
monophosphate
5-
Phosphoribosylamine
Pyrophosphate
L-Glutamic acid
L-Glutamine
Water
Adenosine
triphosphate
Glycine
Adenosine
diphosphate
Phosphate
Glycineamideribotide
10-
Formyltetrahydrofolate
Tetrahydrofolic
acid
5'-
Phosphoribosyl-
N-
formylglycinamide
Adenosine
triphosphate
Water
Adenosine
diphosphate
Phosphoric acid
Phosphoribosylformylglycineamidine
5-amino-1-(5-
phospho-D-
ribosyl)imidazole-
4-carboxylate
5-
Aminoimidazole
ribonucleotide
Carbon dioxide
Adenosine
triphosphate
L-Aspartic acid
Adenosine
diphosphate
Phosphoric acid
SAICAR
Fumaric acid
AICAR
10-
Formyltetrahydrofolate
Tetrahydrofolic
acid
Phosphoribosyl
formamidocarboxamide
Inosinic acid
Water
Water
Pyrophosphate
Adenosine
triphosphate
Xanthylic acid
Ammonia
Adenosine
monophosphate
Pyrophosphate
NAD
Water
NADH
Ammonia
NADP
NADPH
Water
Xanthosine
Phosphate
Phosphate
Ribose
1-phosphate
Water
Inosine
Phosphate
Pyrophosphate
Phosphoribosyl
pyrophosphate
Phosphate
Ribose
1-phosphate
Pyrophosphate
Oxygen
Water
Hydrogen
peroxide
NAD
Water
Uric acid
NADH
Deoxyinosine
Phosphate
Deoxyribose
1-phosphate
Guanosine
triphosphate
L-Aspartic acid
Guanosine
diphosphate
Phosphate
Adenylsuccinic
acid
Adenosine
monophosphate
Water
Ammonia
Fumaric acid
Adenosine
Water
Ammonia
Phosphate
Adenine
Ribose
1-phosphate
Deoxyadenosine
Phosphate
Deoxyribose
1-phosphate
Water
Ammonia
Pyrophosphate
Phosphoribosyl
pyrophosphate
Adenosine
diphosphate
Water
Phosphoric acid
Adenosine
triphosphate
Water
Phosphoric acid
cAMP
Water
Pyrophosphate
Adenosine
triphosphate
Deoxyadenosine
monophosphate
Adenosine
diphosphate
dADP
Water
Phosphoric acid
Adenosine
triphosphate
Adenosine
diphosphate
Deoxyadenosine
triphosphate
Guanosine
2',3'-cyclic
phosphate
Pyrophosphate
Water
IDP
Water
Phosphate
Inosine
triphosphate
Water
Pyrophosphate
Adenosine
triphosphate
Adenosine
diphosphate
Water
Phosphate
Pyrophosphate
5-
Aminoimidazole-
4-carboxamide
Phosphoribosyl
pyrophosphate
Adenosine
triphosphate
Adenosine
diphosphate
Phosphate
Water
Phosphate
Water
Water
Xanthine
Hypoxanthine
Hypoxanthine
Pentose
Phosphate
Pathway
SLC23A1
NUDT2
NME6
ENTPD5
ENTPD8
GUK1
NME6
GUK1
NT5C2
DGUOK
APRT
NT5C2
PNP
HPRT1
PNP
GDA
NUDT5
PRPS1L1
PPAT
GART
GART
PFAS
PAICS
PAICS
ADSL
ATIC
ATIC
ITPA
GMPS
IMPDH1
GMPR
NT5C2
PNP
NT5C2
HPRT1
PNP
HPRT1
XDH
XDH
PNP
ADSS
AMPD1
ADSL
ADA
PNP
PNP
ADA
APRT
ENTPD5
AK1
ATAD1
PDE4D
ADCY2
AK1
NT5C2
NME6
GUCY1A2
GUCY1B3
PDE10A
ENTPD8
ITPA
NME6
ENTPD8
APRT
GART
TXN
TXN
RRM1
RRM2
ENTPD5
TXN
TXN
RRM1
RRM2
PRPP
Xanthin
Guan2P4
H2 O
GTP
GSMP
ATP
GDP
ADP
H2 O
GSMP
Pi
H2 O
Pi
ATP
ADP
ATP
dGDP
ADP
dGTP
ATP
dGMP
ADP
H2 O
doxyG
Pi
ATP
ADP
PPi
Guanine
PRPP
H2 O
Guo
Pi
Pi
Rib-1P
PPi
PRPP
Pi
D1P
H2 O
NH3
ADP-Rib
H2 O
AMP
DRib-5P
ATP
AMP
5-PPRSA
PPi
Glu
Gln
H2 O
ATP
Gly
ADP
Pi
Glyadbt
10-FTHF
THFA
5PPNFG
ATP
H2 O
ADP
Pi
FGAM
CR
5-AnRbn
CO2
ATP
Asp
ADP
Pi
SAICAR
Fumarat
AICAR
10-FTHF
THFA
FosbFca
Imp
H2 O
H2 O
PPi
ATP
XntylcA
NH3
AMP
PPi
NAD
H2 O
NADH
NH3
NADP
NADPH
H2 O
Xanthos
Pi
Pi
Rib-1P
H2 O
Inosine
Pi
PPi
PRPP
Pi
Rib-1P
PPi
O2
H2 O
H2 O2
NAD
H2 O
Urate
NADH
D-Ino
Pi
D1P
GTP
Asp
GDP
Pi
Asuca
AMP
H2 O
NH3
Fumarat
Adenosi
H2 O
NH3
Pi
Adenine
Rib-1P
DA
Pi
D1P
H2 O
NH3
PPi
PRPP
ADP
H2 O
Pi
ATP
H2 O
Pi
cAMP
H2 O
PPi
ATP
dAMP
ADP
dADP
H2 O
Pi
ATP
ADP
doATP
23CGMP
PPi
H2 O
IDP
H2 O
Pi
Ino3P
H2 O
PPi
ATP
ADP
H2 O
Pi
PPi
5Ai4Ca
PRPP
ATP
ADP
Pi
H2 O
Pi
H2 O
H2 O
Ca2+
Mg2+
Zinc
Mg2+
Mg2+
Mg2+
K+
FAD
FAD
Mg2+
Zinc
Zinc
Zinc
Ca2+
Mg2+
Mg2+
Mg2+
Mg2+
Heme
Mg2+
Ca2+
Disulfi
Ca2+
Mg2+
Disulfi
Xanthin
Hyp
Hyp
PW000055
Mg2+
Mg2+
Mg2+
Mg2+
Mg2+
Mg2+
Mg2+
Mg2+
Zinc
Zinc
Mg2+
Ca2+
Mg2+
Fe3+
Fe3+
Peroxisome
Mg2+
Ca2+
Endoplasmic Reticulum
Endoplasmic Reticulum
Peroxisome
Intracellular Space
Extracellular Space
Mitochondria
Cytosol
Mitochondria
Symptoms include arthralgia,
hematuria, mental
retardation, stomatisis, and
urolithiasis.
Kidney
SLC23A1
NUDT2
NME6
ENTPD5
ENTPD8
GUK1
NME6
GUK1
NT5C2
DGUOK
APRT
NT5C2
PNP
HPRT1
PNP
GDA
NUDT5
PRPS1L1
PPAT
GART
GART
PFAS
PAICS
PAICS
ADSL
ATIC
ATIC
ITPA
GMPS
IMPDH1
GMPR
NT5C2
PNP
NT5C2
HPRT1
PNP
HPRT1
XDH
XDH
PNP
ADSS
AMPD1
ADSL
ADA
PNP
PNP
ADA
APRT
ENTPD5
AK1
ATAD1
PDE4D
ADCY2
AK1
NT5C2
NME6
GUCY1A2
GUCY1B3
PDE10A
ENTPD8
ITPA
NME6
ENTPD8
APRT
GART
TXN
TXN
RRM1
RRM2
ENTPD5
TXN
TXN
RRM1
RRM2
PRPP
Xanthin
Guan2P4
H2O
GTP
GSMP
ATP
GDP
ADP
H2O
GSMP
Pi
H2O
Pi
ATP
ADP
ATP
dGDP
ADP
dGTP
ATP
dGMP
ADP
H2O
doxyG
Pi
ATP
ADP
Ppi
Guanine
PRPP
H2O
Guo
PhosfrA
Pi
Rib-1P
Ppi
PRPP
Pi
D1P
H2O
NH3
ADP-Rib
H2O
AMP
DRib-5P
ATP
AMP
5-PPRSA
Ppi
Glu
Gln
H2O
ATP
Gly
ADP
Pi
Glyadbt
10-FTHF
THFA
5PPNFG
ATP
H2O
ADP
PhosfrA
FGAM
CR
5-AnRbn
CO2
ATP
Asp
ADP
PhosfrA
SAICAR
Fumarat
AICAR
10-FTHF
THFA
FosbFca
Imp
H2O
H2O
Ppi
ATP
XntylcA
NH3
AMP
Ppi
NAD
H2O
NADH
NH3
NADP
NADPH
H2O
Xanthos
Pi
Pi
Rib-1P
H2O
Inosine
Pi
Ppi
PRPP
Pi
Rib-1P
Ppi
O2
H2O
H2O2
NAD
H2O
Urate
NADH
D-Ino
Pi
D1P
GTP
Asp
GDP
Pi
Asuca
AMP
H2O
NH3
Fumarat
Adenosi
H2O
NH3
Pi
Adenine
Rib-1P
DA
Pi
D1P
H2O
NH3
Ppi
PRPP
ADP
H2O
PhosfrA
ATP
H2O
PhosfrA
cAMP
H2O
Ppi
ATP
dAMP
ADP
dADP
H2O
PhosfrA
ATP
ADP
doATP
23CGMP
Ppi
H2O
IDP
H2O
Pi
Ino3P
H2O
Ppi
ATP
ADP
H2O
Pi
Ppi
5Ai4Ca
PRPP
ATP
ADP
Pi
H2O
Pi
H2O
H2O
Xanthin
Hyp
Hyp
PW000055