17215925
ChemSpider
1.0
C9H13N5O3
Dihydrobiopterin
239.10184
389682
ChemSpider
SMILES
NC1=NC(=O)C2=C(NCC(=N2)C(O)C(O)CO)N1
937
ChemSpider
389683
ChemSpider
439153
PubChem-compound
SMILES
O
Unknown
HMDB0002275
HMDB
1.0
P30793
UniProt
1125
PubChem-compound
1.0
C04244
KEGG Compound
PW000052
PathWhiz
CHEBI:58104
ChEBI
C9H11N5O3
Sepiapterin
237.08618
247
ChemSpider
20574-65-6
CAS
CHEBI:15846
ChEBI
HMDB0001195
HMDB
53-59-8
CAS
1-hydroxy-2-Oxopropyl tetrahydropterin
TransportCatalysis1583
ACTIVATION
P00374
UniProt
6-Lactoyltetrahydropterin
65253
PubChem-compound
958
ChemSpider
NADH
2,5-Diamino-6-(5'-triphosphoryl-3',4'-trihydroxy-2'-oxopentyl)-amino-4-oxopyrimidine
440841
PubChem-compound
CHEBI:16474
ChEBI
440840
PubChem-compound
1.0
GO:0005829
GENE ONTOLOGY
278
ChemSpider
SMILES
NC1=NC2=C(N=C(C=N2)C(O)C(O)CO)C(=O)N1
Purine Metabolism
SubPathway
6-LACTOYL-5678-TETRAHYDROPTERIN
BioCyc
2009-64-5
CAS
Sepiapterin reductase
TransportCatalysis1581
ACTIVATION
Guanosine triphosphate
SubPathwayOutput
P16152
UniProt
CHEBI:16908
ChEBI
C9H15N5O3
Tetrahydrobiopterin
241.1175
1.0
13846017
PubChem-compound
C9H11N5O3
Dyspropterin
237.08618
Dihydrofolate reductase
Dyspropterin
1.0
6830
PubChem-compound
DIHYDRONEOPTERIN-P3
BioCyc
252
PubChem-compound
Dihydroneopterin triphosphate
169508
PubChem-compound
C21H30N7O17P3
NADPH
745.0911
C21H28N7O17P3
NADP
743.07544
HMDB0002065
HMDB
Dyspropterin
CHEBI:929
ChEBI
HMDB0013642
HMDB
NADPH
BioCyc
C10H16N5O14P3
Guanosine triphosphate
522.99066
1.0
C05926
KEGG Compound
Cytosol
C05923
KEGG Compound
C05922
KEGG Compound
148245
ChemSpider
1.0
58-68-4
CAS
P15121
UniProt
H5O10P3
PPPi
257.90955
HMDB0000217
HMDB
33405-80-0
CAS
10.0
C00006
KEGG Compound
Dihydropteridine reductase
CHEBI:18009
ChEBI
C00001
KEGG Compound
SMILES
[H][C@@]1(CNC2=C(N1)C(=O)N=C(N)N2)[C@@H](O)[C@H](C)O
1.0
SMILES
CC(=O)C(=O)C1CNC2=C(N1)C(=O)NC(N)=N2
1.0
4300
ChemSpider
Dihydroneopterin triphosphate
C00005
KEGG Compound
C00004
KEGG Compound
NADPH
C00003
KEGG Compound
3.5.4.16
false
3.5.4.16
2,5-Diaminopyrimidine nucleoside triphosphate → 2,5-Diamino-6-(5'-triphosphoryl-3',4'-trihydroxy-2'-oxopentyl)-amino-4-oxopyrimidine
LEFT_TO_RIGHT
SMILES
NC(=O)C1=C[N+](=CC=C1)[C@@H]1O[C@H](COP([O-])(=O)OP(O)(=O)OC[C@H]2O[C@H]([C@H](OP(O)(O)=O)[C@@H]2O)N2C=NC3=C2N=CN=C3N)[C@@H](O)[C@H]1O
SMP0000050
SMPDB
HMDB0000221
HMDB
Dihydroneopterin triphosphate
3.5.4.16
false
3.5.4.16
Formamidopyrimidine nucleoside triphosphate + Water → 2,5-Diaminopyrimidine nucleoside triphosphate + Formic acid
LEFT_TO_RIGHT
SMILES
NC(=O)C1=CN(C=CC1)[C@@H]1O[C@H](COP(O)(=O)OP(O)(=O)OC[C@H]2O[C@H]([C@H](OP(O)(O)=O)[C@@H]2O)N2C=NC3=C2N=CN=C3N)[C@@H](O)[C@H]1O
GO:0005759
GENE ONTOLOGY
108735
ChemSpider
ReactionCatalysis2189
ACTIVATION
Hyperphenylalaninemia Due to Guanosine Triphosphate Cyclohydrolase Deficiency
Tetrahydrobiopterin
53-57-6
CAS
CHEBI:17248
ChEBI
C21H27N7O14P2
NAD
663.10913
Dihydropteridine reductase
SubPathwayInteraction815
SubPathwayReaction
SubPathway815Reaction
HMDB0000902
HMDB
CHEBI:804632
ChEBI
CHEBI:17001
ChEBI
89687-39-8
CAS
NAD(P)
BioCyc
C21H29N7O14P2
NADH
665.12476
CHEBI:39949
ChEBI
C9H13N5O3
6-Lactoyltetrahydropterin
239.10184
HMDB0000238
HMDB
GTP cyclohydrolase 1 feedback regulatory protein
114280
ChemSpider
389753
ChemSpider
CHEBI:15996
ChEBI
53-84-9
CAS
SMILES
CC(=O)C(O)C1CNC2=C(N1)C(=O)NC(N)=N2
Dyspropterin
C00272
KEGG Compound
GO:0005737
GENE ONTOLOGY
1.0
Carbonyl reductase [NADPH] 1
GTP cyclohydrolase
Q03393
UniProt
CHEBI:17804
ChEBI
GTP cyclohydrolase 1 feedback regulatory protein
NADP
ReactionCatalysis795
ACTIVATION
PW000463
PathWhiz
ReactionCatalysis796
ACTIVATION
58746
ChemSpider
ReactionCatalysis793
ACTIVATION
ReactionCatalysis794
ACTIVATION
ReactionCatalysis792
ACTIVATION
6-pyruvoyl tetrahydrobiopterin synthase
P30047
UniProt
C00835
KEGG Compound
CPD-374
BioCyc
Sepiapterin reductase
SMILES
NC(=O)C1=C[N+](=CC=C1)[C@@H]1O[C@H](COP([O-])(=O)OP(O)(=O)OC[C@H]2O[C@H]([C@H](O)[C@@H]2O)N2C=NC3=C2N=CN=C3N)[C@@H](O)[C@H]1O
Formamidopyrimidine nucleoside triphosphate
6-pyruvoyl tetrahydrobiopterin synthase
C04874
KEGG Compound
5682
ChemSpider
3.5.4.16
false
3.5.4.16
Guanosine triphosphate + Water → Formamidopyrimidine nucleoside triphosphate + Formic acid
LEFT_TO_RIGHT
SMILES
NC(=O)C1=CN(C=CC1)[C@@H]1O[C@H](CO[P@](O)(=O)O[P@](O)(=O)OC[C@H]2O[C@H]([C@H](O)[C@@H]2O)N2C=NC3=C(N)N=CN=C23)[C@@H](O)[C@H]1O
Formic acid
SMILES
CC(O)C(=O)C1CNC2=C(N1)C(=O)NC(N)=N2
TETRA-H-BIOPTERIN
BioCyc
10380-08-2
CAS
SMP0000487
SMPDB
C9H11N5O4
Neopterin
253.0811
1.0
Mitochondrial Matrix
BIOPTERIN
BioCyc
1218-98-0
CAS
Carbonyl reductase [NADPH] 1
2.0
C00044
KEGG Compound
Dihydrofolate reductase
5675
ChemSpider
C06148
KEGG Compound
4.2.3.12
false
4.2.3.12
Dihydroneopterin triphosphate → Dyspropterin + PPPi
LEFT_TO_RIGHT
HMDB0000027
HMDB
1.0
1.1.1.153
false
1.1.1.153
6-Lactoyltetrahydropterin + NAD ↔ 1-hydroxy-2-Oxopropyl tetrahydropterin + NADH
REVERSIBLE
18971002
PubChem-compound
Cytoplasm
HMDB0000142
HMDB
10.0
Homo sapiens
6-1S2R-12-DIHYDROXY-3-TRIPHOSPHOOXY
BioCyc
CHEBI:18372
ChEBI
SMILES
C[C@H](O)[C@H](O)C1=NC2=C(NC1)NC(N)=NC2=O
7732-18-5
CAS
388299
ChemSpider
C03684
KEGG Compound
C00058
KEGG Compound
1.0
C04895
KEGG Compound
HMDB0001487
HMDB
CH2O2
Formic acid
46.005478
1.0
Dihydrofolate reductase
HMDB0000038
HMDB
C9H18N5O14P3
2,5-Diaminopyrimidine nucleoside triphosphate
513.0063
C10H18N5O15P3
Formamidopyrimidine nucleoside triphosphate
541.0012
Dihydrobiopterin
FORMATE
BioCyc
Water
NAD
C9H16N5O13P3
Dihydroneopterin triphosphate
494.99576
659
PubChem-compound
C9H18N5O14P3
2,5-Diamino-6-(5'-triphosphoryl-3',4'-trihydroxy-2'-oxopentyl)-amino-4-oxopyrimidine
513.0063
Transport10414
false
Dyspropterin (←) Transport: Homo sapiens, Cell, Mitochondrial Matrix to Homo sapiens, Cell, Cytoplasm
LEFT_TO_RIGHT
1.0
Neopterin
1093
ChemSpider
Transport10416
false
Dihydroneopterin triphosphate (←) Transport: Homo sapiens, Cell, Cytoplasm to Homo sapiens, Cell, Mitochondrial Matrix
LEFT_TO_RIGHT
HMDB0006821
HMDB
HMDB0006822
HMDB
121885
PubChem-compound
HMDB0006823
HMDB
1.0
1.0
ReactionCatalysis799
ACTIVATION
SMILES
C[C@H](O)C(=O)C1=NC2=C(NC1)NC(N)=NC2=O
1.0
ReactionCatalysis797
ACTIVATION
ReactionCatalysis798
ACTIVATION
9606
TAXONOMY
962
PubChem-compound
17528-72-2
CAS
HMDB0000845
HMDB
5893
PubChem-compound
SMILES
NC1=NC(N[C@@H]2O[C@H](COP(O)(=O)OP(O)(=O)OP(O)(O)=O)[C@@H](O)[C@H]2O)=C(N)C(=O)N1
SMILES
NC1=NC(N[C@@H]2O[C@H](COP(O)(=O)OP(O)(=O)OP(O)(O)=O)[C@@H](O)[C@H]2O)=C(NC=O)C(=O)N1
CHEBI:30751
ChEBI
SMILES
NC1=NC(=O)C(N)=C(NCC(=O)C(O)C(O)COP(O)(=O)OP(O)(=O)OP(O)(O)=O)N1
6-PYRUVOYL-5678-TETRAHYDROPTERIN
BioCyc
HMDB0002111
HMDB
SMILES
OC=O
CHEBI:15377
ChEBI
Unknown
128973
PubChem-compound
C00404
KEGG Compound
P09417
UniProt
1.0
6569
ChemSpider
HMDB0012282
HMDB
5886
PubChem-compound
7,8-Dihydroneopterin
Dihydropteridine reductase
GTP
BioCyc
CHEBI:15372
ChEBI
SMILES
NC1=NC2=C(N=C(CN2)[C@H](O)[C@H](O)COP(O)(=O)OP(O)(=O)OP(O)(O)=O)C(=O)N1
Reaction219018
false
Dihydrobiopterin + NADPH → NADP + Tetrahydrobiopterin
LEFT_TO_RIGHT
HMDB0000980
HMDB
C9H13N5O3
1-hydroxy-2-Oxopropyl tetrahydropterin
239.10184
HMDB0001273
HMDB
Aldose reductase
CHEBI:64277
ChEBI
GTP cyclohydrolase 1
983
PubChem-compound
Aldose reductase
SMILES
NC1=NC2=C(N=CN2[C@@H]2O[C@H](COP(O)(=O)OP(O)(=O)OP(O)(O)=O)[C@@H](O)[C@H]2O)C(=O)N1
440923
PubChem-compound
639
ChemSpider
64-18-6
CAS
C02953
KEGG Compound
C9H13N5O4
7,8-Dihydroneopterin
255.09676
1.0
26777299
ChemSpider
6779-87-9
CAS
1.5.1.3
false
1.5.1.3
7,8-Dihydroneopterin + NADPH ← NADP + Tetrahydrobiopterin + Water
RIGHT_TO_LEFT
ReactionCatalysis208477
ACTIVATION
GTP cyclohydrolase 1
22833512
PubChem-compound
Reaction1111
false
7,8-Dihydroneopterin → Neopterin
LEFT_TO_RIGHT
SMILES
OP(O)(=O)OP(O)(=O)OP(O)(O)=O
P35270
UniProt
H2O
Water
18.010565
ReactionCatalysis805
ACTIVATION
PPPi
ReactionCatalysis803
ACTIVATION
2,5-Diaminopyrimidine nucleoside triphosphate
ReactionCatalysis804
ACTIVATION
ReactionCatalysis801
ACTIVATION
NADH
BioCyc
86-01-1
CAS
ReactionCatalysis800
ACTIVATION
NAD
BioCyc
1.1.1.153
false
1.1.1.153
6-Lactoyltetrahydropterin + NADP → NADPH + Tetrahydrobiopterin
LEFT_TO_RIGHT
1.0
4455
PubChem-compound
P3I
BioCyc
3.5.4.16
false
3.5.4.16
2,5-Diamino-6-(5'-triphosphoryl-3',4'-trihydroxy-2'-oxopentyl)-amino-4-oxopyrimidine → Dihydroneopterin triphosphate + Water
LEFT_TO_RIGHT
1.0
2.0
1.0
Reaction1108
false
NADP + Sepiapterin ← 7,8-Dihydroneopterin + NADPH
RIGHT_TO_LEFT
Reaction1105
false
6-Lactoyltetrahydropterin → Sepiapterin
LEFT_TO_RIGHT
17094-01-8
CAS
1.1.1.153
false
1.1.1.153
NADPH + Sepiapterin ← 7,8-Dihydroneopterin + NADP
RIGHT_TO_LEFT
1.1.1.21
false
1.1.1.21
Dyspropterin + NADP → 6-Lactoyltetrahydropterin + NADPH
LEFT_TO_RIGHT
Reaction1104
false
Dyspropterin + NADP → 6-Lactoyltetrahydropterin + NADPH
LEFT_TO_RIGHT
1.1.1.153
false
1.1.1.153
Dyspropterin + NADP → 1-hydroxy-2-Oxopropyl tetrahydropterin + NADPH
LEFT_TO_RIGHT
1.1.1.153
false
1.1.1.153
Dyspropterin + NADP → 6-Lactoyltetrahydropterin + NADPH
LEFT_TO_RIGHT
Sepiapterin