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DraconiticBiochemistryBiomolecules · Catabolism · Biosynthesis · MetabolismMap
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4a-hydroxytetrahydrobiopterin => q-dihydrobiopterin + H2O

4aOH-BH4 → qDHB + H2O

Reactome
R-HSA-71146
Compartment
cytosol
Rhea
RHEA:11921

Inputs

Outputs

Catalysed by

  • 4-alpha-hydroxytetrahydrobiopterin dehydratase activity of PCBD1 tetramer [cytosol]EC 4.2.1.96

Literature

  1. Mutation in the 4a-carbinolamine dehydratase gene leads to mild hyperphenylalaninemia with defective cofactor metabolism. Am J Hum Genet, 1993
  2. Three-dimensional structure of the bifunctional protein PCD/DCoH, a cytoplasmic enzyme interacting with transcription factor HNF1. EMBO J, 1995
  3. 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