Reactome | 4a-hydroxytetrahydrobiopterin => q-dihydrobiopterin + H2O

4a-hydroxytetrahydrobiopterin => q-dihydrobiopterin + H2O

Stable Identifier

R-HSA-71146

Type

Reaction [transition]

Species

Homo sapiens

Compartment

cytosol

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4a-hydroxytetrahydrobiopterin => q-dihydrobiopterin + H2O

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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).

Literature References

PubMed ID	Title	Journal	Year
8352282	Mutation in the 4a-carbinolamine dehydratase gene leads to mild hyperphenylalaninemia with defective cofactor metabolism. Citron, BA, Kaufman, S, Milstien, S, Naylor, EW, Greene, CL, Davis, MD	Am J Hum Genet	1993
8444860	Phenylalanine hydroxylase-stimulating protein/pterin-4 alpha-carbinolamine dehydratase from rat and human liver. Purification, characterization, and complete amino acid sequence. Hauer, CR, Rebrin, I, Thony, B, Neuheiser, F, Curtius, HC, Hunziker, P, Blau, N, Ghisla, S, Heizmann, CW	J Biol Chem	1993
7744010	Three-dimensional structure of the bifunctional protein PCD/DCoH, a cytoplasmic enzyme interacting with transcription factor HNF1. Ficner, R, Sauer, UH, Stier, G, Suck, D	EMBO J	1995