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Proteins/UNIPROT:Q9H0N5
Plays a role in tetrahydrobiopterin biosynthesis which is a cofactor for amino acid hydroxylases. May prevent both the formation of 7-pterins and accelerate the formation of quinonoid-BH2 (By similarity). Regulates the dimerization of homeodomain protein HNF1A and enhances its transcriptional activity. Also acts as a coactivator for HNF1B-dependent transcription..
Function from UniProtKB (Q9H0N5), read 2026-09-23. CC BY 4.0.
Curated compounds routed: 0
41 statements
41 statements · 4 reference databases · Subject: PCBD2 · Sources: gene ontology, Hetionet, Human-GEM, Rhea
8 reactions
4a-hydroxytetrahydrobiopterinwater+MAM02632c
Reaction pageReversibleBalancedEC 4.2.1.96cytosol
Human-GEM MAR04539CC BY 4.0Reference data
4a-hydroxytetrahydrobiopterinwater+MAM02632n
Reaction pageReversibleBalancedEC 4.2.1.96nucleus
Human-GEM MAR04540CC BY 4.0
7 names
Resolves to: PCBD2
MAM02632cMAM02823c
Reaction pageReversibleBalancedEC 4.2.1.96cytosol
Human-GEM MAR04541CC BY 4.0Reference data
MAM02632nMAM02823n
Reaction pageReversibleBalancedEC 4.2.1.96nucleus
Human-GEM MAR04542CC BY 4.0Reference data
4a-hydroxytetrahydrobiopterinwater+MAM02823c
Reaction pageReversibleBalancedEC 4.2.1.96cytosol
Human-GEM MAR04543CC BY 4.0Reference data
4a-hydroxytetrahydrobiopterinwater+MAM02823n
Reaction pageReversibleBalancedEC 4.2.1.96nucleus
Human-GEM MAR04544CC BY 4.0Reference data
4a-hydroxytetrahydrobiopterinMAM01698c+water
Reaction pageLeft to rightBalancedEC 4.2.1.96cytosol
Human-GEM MAR08538CC BY 4.0Reference data
(4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin(6R)-L-erythro-6,7-dihydrobiopterin+water
Reaction pageDirection not assertedBalanced
Rhea RHEA:11920CC BY 4.0Reference data