4a-hydroxytetrahydrobiopterin => q-dihydrobiopterin + H2O

Stable Identifier
R-HSA-71146
Type
Reaction [transition]
Species
Homo sapiens
Compartment
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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
Participants
Participant Of
Catalyst Activity
Catalyst Activity
Title
4-alpha-hydroxytetrahydrobiopterin dehydratase activity of PCBD1 tetramer [cytosol]
Physical Entity
Activity
Orthologous Events
Authored