Biosynthesis and regeneration of tetrahydrobiopterin and catabolism of phenylalanine (WP4156)

New PW, Chapter 1 of Blau (physicians guide to... inheritant metabolic diseases.
last edited


DeSl, Egonw, IreneHemel, Ddigles, Josienlandman, MaintBot, Fehrhart, and Finterly

Cited In

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Homo sapiens


Inborn Errors of Metabolism (IEM) Pathways Rare Diseases


Disease Ontology: aromatic L-amino acid decarboxylase deficiency sepiapterin reductase deficiency BH4-deficient hyperphenylalaninemia A dystonia 5 megaloblastic anemia

Pathway Ontology: tetrahydrobiopterin metabolic pathway Segawa syndrome pathway phenylalanine degradation pathway


Label Type Compact Identifier
DHPR Protein wikidata:Q21145902
Primapterin Metabolite wikidata:Q26261687
DHFR Protein wikidata:Q417149
q-BH2 Metabolite chebi:43120
BH4 Metabolite chebi:30436
PTPS GeneProduct uniprot:Q03393
GTPCH GeneProduct uniprot:P30793
PTP Metabolite chebi:136564
Phe Metabolite chebi:28044
Serotonin Metabolite chebi:28790
HO-BH4 Metabolite chebi:15642
SR GeneProduct wikidata:Q15331000
5-OH-Trp Metabolite chebi:58266
Dopamine Metabolite wikidata:Q170304
HVA Metabolite wikidata:Q903566
sepiapterin Metabolite wikidata:Q2271580
Biopterin Metabolite wikidata:Q41793745
L-DOPA Metabolite wikidata:Q300989
NH2TP Metabolite chebi:58462
TH Protein Wikidata
GTP Metabolite ChEBI
5HIAA Metabolite HMDB
oxo-PH4 Metabolite None
Phenyl-alanine Metabolite Wikidata
TPH Protein Enzyme Nomenclature
Neopterin Metabolite Wikidata
Trp Metabolite Wikidata
PCD Protein Enzyme Nomenclature
7,8-BH2 Metabolite Wikidata
AADC Protein Wikidata
CR Protein Wikidata
tyr Metabolite Wikidata
PAH Protein Wikidata
O2 Metabolite ChEBI
O2 Metabolite ChEBI
SR GeneProduct Wikidata
SR GeneProduct Wikidata


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