4a-hydroxytetrahydrobiopterin => q-dihydrobiopterin + H2O
4aOH-BH4 → qDHB + H2O
- Reactome
- R-HSA-71146
- Compartment
- cytosol
- Rhea
- RHEA:11921
Inputs
- 4aOH-BH4CHEBI:15642, C9H15N5O4, cytosol
Outputs
- qDHBCHEBI:43120, C9H13N5O3, cytosol
- H2OCHEBI:15377, H2O, cytosol
Catalysed by
- 4-alpha-hydroxytetrahydrobiopterin dehydratase activity of PCBD1 tetramer [cytosol]EC 4.2.1.96
Literature
- Mutation in the 4a-carbinolamine dehydratase gene leads to mild hyperphenylalaninemia with defective cofactor metabolism. Am J Hum Genet, 1993
- Three-dimensional structure of the bifunctional protein PCD/DCoH, a cytoplasmic enzyme interacting with transcription factor HNF1. EMBO J, 1995
- Phenylalanine hydroxylase-stimulating protein/pterin-4 alpha-carbinolamine dehydratase from rat and human liver. Purification, characterization, and complete amino acid sequence. J Biol Chem, 1993