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17215925 ChemSpider 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 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 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 Guanosine triphosphate SubPathwayOutput P16152 UniProt CHEBI:16908 ChEBI C9H15N5O3 Tetrahydrobiopterin 241.1175 1.0 13846017 PubChem-compound C9H11N5O3 Dyspropterin 237.08618 Dihydrofolate reductase 1.0 6830 PubChem-compound DIHYDRONEOPTERIN-P3 BioCyc 252 PubChem-compound 169508 PubChem-compound C21H30N7O17P3 NADPH 745.0911 C21H29N7O17P3 NADP 744.08325 HMDB0002065 HMDB 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 CC(O)C(O)C1CNC2=C(N1)C(=O)N=C(N)N2 1.0 SMILES CC(=O)C(=O)C1CNC2=C(N1)C(=O)NC(N)=N2 1.0 4300 ChemSpider 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](CO[P@](O)(=O)O[P@](O)(=O)OC[C@H]2O[C@H]([C@H](OP(O)(O)=O)[C@@H]2O)N2C=NC3=C(N)N=CN=C23)[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=C(N)N=CN=C23)[C@H](O)[C@@H]1O 108735 ChemSpider Hyperphenylalaninemia Due to DHPR-Deficiency ReactionCatalysis2189 ACTIVATION Tetrahydrobiopterin 53-57-6 CAS CHEBI:17248 ChEBI C21H28N7O14P2 NAD 664.11694 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 Carbonyl reductase [NADPH] 1 GTP cyclohydrolase Q03393 UniProt CHEBI:17804 ChEBI GTP cyclohydrolase 1 feedback regulatory protein NADP ReactionCatalysis795 ACTIVATION ReactionCatalysis796 ACTIVATION 58746 ChemSpider ReactionCatalysis793 ACTIVATION ReactionCatalysis794 ACTIVATION ReactionCatalysis792 ACTIVATION SMP0000489 SMPDB 6-pyruvoyl tetrahydrobiopterin synthase PW000465 PathWhiz 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 C9H11N5O4 Neopterin 253.0811 1.0 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 1.1.1.153 false 1.1.1.153 6-Lactoyltetrahydropterin + NAD ↔ 1-hydroxy-2-Oxopropyl tetrahydropterin + NADH REVERSIBLE 18971002 PubChem-compound HMDB0000142 HMDB 10.0 Homo sapiens 6-1S2R-12-DIHYDROXY-3-TRIPHOSPHOOXY BioCyc CHEBI:18372 ChEBI SMILES CC(O)C(O)C1=NC2=C(NC1)N=C(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 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 1.0 Neopterin 1093 ChemSpider 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 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 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 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 ReactionCatalysis806 ACTIVATION PPPi ReactionCatalysis803 ACTIVATION 2,5-Diaminopyrimidine nucleoside triphosphate ReactionCatalysis804 ACTIVATION ReactionCatalysis801 ACTIVATION NADH BioCyc 86-01-1 CAS ReactionCatalysis800 ACTIVATION 1.5.1.34 false 1.5.1.34 NADP + Tetrahydrobiopterin → Dihydrobiopterin + NADPH LEFT_TO_RIGHT 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 GTP cyclohydrolase GTP cyclohydrolase GTP cyclohydrolase GTP cyclohydrolase 6-pyruvoyl tetrahydrobiopterin synthase Sepiapterin reductase Sepiapterin reductase Aldose reductase Carbonyl reductase [NADPH] 1 Sepiapterin reductase Carbonyl reductase [NADPH] 1 Sepiapterin reductase Dihydrofolate reductase Dihydropteridine reductase Sepiapterin reductase Guanosine triphosphate Formamidopyrimidine nucleoside triphosphate H 2 O 2,5-Diaminopyrimidine nucleoside triphosphate Formic acid H 2 O Formic acid 2,5-Diamino-6-(5'- triphosphoryl-3',4'- trihydroxy-2'-oxopentyl)- amino-4-oxopyrimidine Dihydroneopterin triphosphate H 2 O Dyspropterin PPPi NADP NADPH 1-hydroxy-2-Oxopropyl tetrahydropterin NADP 6-Lactoyltetrahydropterin NADPH NADP NADPH Sepiapterin NADPH 7,8-Dihydroneopterin NADP NADP NADPH NADP Tetrahydrobiopterin NADPH NADPH NADP H 2 O Accumulation Neopterin NADP Dihydrobiopterin NADPH NAD NADH Zinc Zinc Zinc Zinc Zinc Purine Metabolism Intracellular space
17215925 ChemSpider 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 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 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 Guanosine triphosphate SubPathwayOutput P16152 UniProt CHEBI:16908 ChEBI C9H15N5O3 Tetrahydrobiopterin 241.1175 1.0 13846017 PubChem-compound C9H11N5O3 Dyspropterin 237.08618 Dihydrofolate reductase 1.0 6830 PubChem-compound DIHYDRONEOPTERIN-P3 BioCyc 252 PubChem-compound 169508 PubChem-compound C21H30N7O17P3 NADPH 745.0911 C21H29N7O17P3 NADP 744.08325 HMDB0002065 HMDB 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 CC(O)C(O)C1CNC2=C(N1)C(=O)N=C(N)N2 1.0 SMILES CC(=O)C(=O)C1CNC2=C(N1)C(=O)NC(N)=N2 1.0 4300 ChemSpider 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](CO[P@](O)(=O)O[P@](O)(=O)OC[C@H]2O[C@H]([C@H](OP(O)(O)=O)[C@@H]2O)N2C=NC3=C(N)N=CN=C23)[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=C(N)N=CN=C23)[C@H](O)[C@@H]1O 108735 ChemSpider Hyperphenylalaninemia Due to DHPR-Deficiency ReactionCatalysis2189 ACTIVATION Tetrahydrobiopterin 53-57-6 CAS CHEBI:17248 ChEBI C21H28N7O14P2 NAD 664.11694 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 Carbonyl reductase [NADPH] 1 GTP cyclohydrolase Q03393 UniProt CHEBI:17804 ChEBI GTP cyclohydrolase 1 feedback regulatory protein NADP ReactionCatalysis795 ACTIVATION ReactionCatalysis796 ACTIVATION 58746 ChemSpider ReactionCatalysis793 ACTIVATION ReactionCatalysis794 ACTIVATION ReactionCatalysis792 ACTIVATION SMP0000489 SMPDB 6-pyruvoyl tetrahydrobiopterin synthase PW000465 PathWhiz 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 C9H11N5O4 Neopterin 253.0811 1.0 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 1.1.1.153 false 1.1.1.153 6-Lactoyltetrahydropterin + NAD ↔ 1-hydroxy-2-Oxopropyl tetrahydropterin + NADH REVERSIBLE 18971002 PubChem-compound HMDB0000142 HMDB 10.0 Homo sapiens 6-1S2R-12-DIHYDROXY-3-TRIPHOSPHOOXY BioCyc CHEBI:18372 ChEBI SMILES CC(O)C(O)C1=NC2=C(NC1)N=C(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 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 1.0 Neopterin 1093 ChemSpider 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 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 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 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 ReactionCatalysis806 ACTIVATION PPPi ReactionCatalysis803 ACTIVATION 2,5-Diaminopyrimidine nucleoside triphosphate ReactionCatalysis804 ACTIVATION ReactionCatalysis801 ACTIVATION NADH BioCyc 86-01-1 CAS ReactionCatalysis800 ACTIVATION 1.5.1.34 false 1.5.1.34 NADP + Tetrahydrobiopterin → Dihydrobiopterin + NADPH LEFT_TO_RIGHT 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 GCH1 GCH1 GCH1 GCH1 PTS SPR SPR AKR1B1 CBR1 SPR CBR1 SPR DHFR QDPR SPR Guanosine triphosphate Formamidopyrimidine nucleoside triphosphate Water 2,5- Diaminopyrimidine nucleoside triphosphate Formic acid Water Formic acid 2,5-Diamino- 6-(5'- triphosphoryl- 3',4'- trihydroxy- 2'- oxopentyl)- amino-4- oxopyrimidine Dihydroneopterin triphosphate Water Dyspropterin PPPi NADP NADPH 1-hydroxy-2- Oxopropyl tetrahydropterin NADP 6- Lactoyltetrahydropterin NADPH NADP NADPH Sepiapterin NADPH 7,8- Dihydroneopterin NADP NADP NADPH NADP Tetrahydrobiopterin NADPH NADPH NADP Water Accumulation Neopterin NADP Dihydrobiopterin NADPH NAD NADH Purine Metabolism