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

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

Authors

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

Cited In

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Organism

Homo sapiens

Communities

Inborn Errors of Metabolism (IEM) Pathways Rare Diseases

Annotations

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

Participants

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
None

References

  1. PubMed Europe PMC Scholia