EC Number   |
Natural Substrates |
|---|
    4.2.1.96 | (6R)-6-(L-erythro-1,2-dihydroxypropyl)-5,6,7,8-tetrahydro-4a-hydroxypterin |
- |
| 4.2.1.96 | more |
- |
| 4.2.1.96 | more |
a defect in 4a-hydroxytetrahydrobiopterin dehydratase provoces the conversion of the 6-substituted pterins to their 7-substituted isomers |
| 4.2.1.96 | more |
both PhhB and phenylalanine hydroxylase (PhhA) are induced coordinately in the presence of either L-tyrosine or L-phenylalanine, but PhhB exhibits a significant basal level of activity that is lacking for PhhA. PhhA and PhhB form a protein-protein complex |
| 4.2.1.96 | more |
dehydratase/DCoHalpha, has the kinetic properties necessary for regenerating tetrahydrobiopterin cofactor for phenylalanine hydroxylase. Properties of dehydratase/DCoHalpha are consistent with the hypothesis that the activity of this isozyme could account for the relatively mild symptoms reported for patients with a defect in dehydratase/DCoH |
| 4.2.1.96 | more |
enzyme plays a role in the regulation of expression of phenylalanine hydroxylase via transcription factor HNF1alpha |
| 4.2.1.96 | more |
the codevelopment of 4a-hydroxytetrahydropterin dehydratase with dihydropteridine reductase strongly supports a physiologically significant role for the dehydratase in tetrahydrobiopterin regeneration |
| 4.2.1.96 | more |
the cytosolic enzyme is involved in the regeneration of tetrahydrobiopterin, the cofactor of aromatic amino acid monooxygenases |
| 4.2.1.96 | more |
the enzyme is essential in vivo to prevent rearrangement of 4a-hydroxy-6(R)-tetrahydrobiopterin and to maintain the supply of tetrahydrobiopterin cofactor for hydroxylases under conditions where the nonenzymatic rate would be inadequate |
| 4.2.1.96 | more |
the enzyme is essentially identical to a cofactor that regulates dimerization of a nuclear homeodomain-containing protein involved in transcription |
