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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
Diguanosine tetraphosphate
H
2
O
Guanosine triphosphate
Guanosine monophosphate
ATP
Guanosine diphosphate
ADP
H
2
O
Guanosine monophosphate
P
i
H
2
O
P
i
ATP
ADP
ATP
dGDP
ADP
dGTP
ATP
2'-Deoxyguanosine
5'-monophosphate
ADP
H
2
O
Deoxyguanosine
P
i
ATP
ADP
PP
i
Guanine
Phosphoribosyl pyrophosphate
H
2
O
Guanosine
P
i
P
i
Ribose 1-phosphate
PP
i
Phosphoribosyl pyrophosphate
P
i
Deoxyribose 1-phosphate
H
2
O
Xanthine
NH
3
Adenosine diphosphate ribose
H
2
O
AMP
D-Ribose 5-phosphate
ATP
AMP
5-Phosphoribosylamine
PP
i
L-Glutamic acid
L-Glutamine
H
2
O
ATP
Glycine
ADP
P
i
Glycineamideribotide
10-Formyltetrahydrofolate
Tetrahydrofolic acid
5'-Phosphoribosyl-N-
formylglycinamide
ATP
H
2
O
ADP
P
i
Phosphoribosylformylglycineamidine
5-amino-1-(5-phospho-D-
ribosyl)imidazole-4-
carboxylate
5-Aminoimidazole
ribonucleotide
CO
2
ATP
L-Aspartic acid
ADP
P
i
SAICAR
Fumaric acid
AICAR
10-Formyltetrahydrofolate
Tetrahydrofolic acid
Phosphoribosyl
formamidocarboxamide
Inosinic acid
H
2
O
H
2
O
PP
i
ATP
Xanthylic acid
NH
3
AMP
PP
i
NAD
H
2
O
NADH
NH
3
NADP
NADPH
H
2
O
Xanthosine
P
i
P
i
Ribose 1-phosphate
H
2
O
Inosine
P
i
PP
i
Phosphoribosyl pyrophosphate
P
i
Hypoxanthine
Ribose 1-phosphate
PP
i
O
2
H
2
O
H
2
O
2
NAD
H
2
O
Uric acid
NADH
Deoxyinosine
P
i
Deoxyribose 1-phosphate
GTP
L-Aspartic acid
GDP
P
i
Adenylsuccinic acid
Adenosine monophosphate
H
2
O
NH
3
Fumaric acid
Adenosine
H
2
O
NH
3
P
i
Adenine
Ribose 1-phosphate
Deoxyadenosine
P
i
Deoxyribose 1-phosphate
H
2
O
NH
3
PP
i
Phosphoribosyl pyrophosphate
Adenosine diphosphate
H
2
O
P
i
Adenosine triphosphate
H
2
O
P
i
cAMP
H
2
O
PP
i
ATP
Deoxyadenosine monophosphate
ADP
dADP
H
2
O
P
i
ATP
ADP
Deoxyadenosine triphosphate
Guanosine 2',3'-cyclic
phosphate
PP
i
H
2
O
IDP
H
2
O
P
i
Inosine triphosphate
H
2
O
PP
i
ATP
ADP
H
2
O
P
i
PP
i
5-Aminoimidazole-4-carboxamide
Phosphoribosyl pyrophosphate
ATP
ADP
P
i
H
2
O
P
i
H
2
O
H
2
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
Pentose
Phosphate
Pathway
Magnesium
Magnesium
Magnesium
Magnesium
Magnesium
Magnesium
Magnesium
Magnesium
Zinc (II) ion
Zinc (II) ion
Magnesium
Calcium
Magnesium
Fe3+
Fe3+
Magnesium
Calcium
Endoplasmic Reticulum
Peroxisome
Endoplasmic Reticulum
Peroxisome
Intracellular Space
Extracellular Space
Mitochondria
Cytosol
Mitochondria
Symptoms commonly include
fatigue, myalgia (i.e.
muscle pain) and cramps
after exercise.
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
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
Xanthine
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
Hypoxanthine
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
Pentose
Phosphate
Pathway
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
Guan2P4
H
2
O
GTP
GSMP
ATP
GDP
ADP
H
2
O
GSMP
P
i
H
2
O
P
i
ATP
ADP
ATP
dGDP
ADP
dGTP
ATP
dGMP
ADP
H
2
O
doxyG
P
i
ATP
ADP
PP
i
Guanine
PRPP
H
2
O
Guo
P
i
P
i
Rib-1P
PP
i
PRPP
P
i
D1P
H
2
O
Xanthin
NH
3
ADP-Rib
H
2
O
AMP
DRib-5P
ATP
AMP
5-PPRSA
PP
i
Glu
Gln
H
2
O
ATP
Gly
ADP
P
i
Glyadbt
10-FTHF
THFA
5PPNFG
ATP
H
2
O
ADP
P
i
FGAM
CR
5-AnRbn
CO
2
ATP
Asp
ADP
P
i
SAICAR
Fumarat
AICAR
10-FTHF
THFA
FosbFca
Imp
H
2
O
H
2
O
PP
i
ATP
XntylcA
NH
3
AMP
PP
i
NAD
H
2
O
NADH
NH
3
NADP
NADPH
H
2
O
Xanthos
P
i
P
i
Rib-1P
H
2
O
Inosine
P
i
PP
i
PRPP
P
i
Hyp
Rib-1P
PP
i
O
2
H
2
O
H
2
O
2
NAD
H
2
O
Urate
NADH
D-Ino
P
i
D1P
GTP
Asp
GDP
P
i
Asuca
AMP
H
2
O
NH
3
Fumarat
Adenosi
H
2
O
NH
3
P
i
Adenine
Rib-1P
DA
P
i
D1P
H
2
O
NH
3
PP
i
PRPP
ADP
H
2
O
P
i
ATP
H
2
O
P
i
cAMP
H
2
O
PP
i
ATP
dAMP
ADP
dADP
H
2
O
P
i
ATP
ADP
doATP
23CGMP
PP
i
H
2
O
IDP
H
2
O
P
i
Ino3P
H
2
O
PP
i
ATP
ADP
H
2
O
P
i
PP
i
5Ai4Ca
PRPP
ATP
ADP
P
i
H
2
O
P
i
H
2
O
H
2
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
PW000055
Mg2+
Mg2+
Mg2+
Mg2+
Mg2+
Mg2+
Mg2+
Mg2+
Zinc
Zinc
Mg2+
Ca2+
Mg2+
Fe3+
Fe3+
Mg2+
Ca2+
Endoplasmic Reticulum
Peroxisome
Endoplasmic Reticulum
Peroxisome
Intracellular Space
Extracellular Space
Mitochondria
Cytosol
Mitochondria
Symptoms commonly include
fatigue, myalgia (i.e.
muscle pain) and cramps
after exercise.