Reaction: 4a-hydroxytetrahydrobiopterin => q-dihydrobiopterin + H2O
- in pathway: Phenylalanine metabolism
Cytosolic pterin-4-alpha-carbinolamine dehydratase (PCDB1) catalyzes the reaction of 4a-hydroxytetrahydrobiopterin to form q-dihydrobiopterin and water (Hauer et al. 1993. The active enzyme is a homotetramer (Ficner et al. 1995); mutations in the PCDB1 gene are associated with mild hyperphenylalanemia in vivo (Citron et al. 1993).
Reaction - small molecule participants:
H2O [cytosol]
qDHB [cytosol]
4aOH-BH4 [cytosol]
Reactome.org reaction link: R-HSA-71146
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Reaction input - small molecules:
4a-hydroxytetrahydrobiopterin
Reaction output - small molecules:
water
6,7-dihydrobiopterin
Reactome.org link: R-HSA-71146