4.2.1.96: 4a-hydroxytetrahydrobiopterin dehydratase
This is an abbreviated version!
For detailed information about 4a-hydroxytetrahydrobiopterin dehydratase, go to the full flat file.
Word Map on EC 4.2.1.96
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4.2.1.96
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pterins
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hyperphenylalaninemias
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dihydropteridine
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bh4
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hydroxylases
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cyclohydrolase
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7-biopterin
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6-pyruvoyl-tetrahydropterin
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vitiligo
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hnf1alpha
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sepiapterin
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tetrahydropterins
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depigmentation
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gtpch
- 4.2.1.96
- pterins
- hyperphenylalaninemias
- dihydropteridine
- bh4
- hydroxylases
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cyclohydrolase
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7-biopterin
- 6-pyruvoyl-tetrahydropterin
- vitiligo
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hnf1alpha
- sepiapterin
- tetrahydropterins
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depigmentation
- gtpch
Reaction
Synonyms
4 alpha-Hydroxy-tetrahydropterin dehydratase, 4-alpha-hydroxy-tetrahydropterin dehydratase, 4-alpha-hydroxytetrahydrobiopterin dehydratase, 4a-Carbinolamine dehydratase, 4a-Hydroxytetrahydrobiopterin dehydratase, 4a-Hydroxytetrahydropterin dehydratase, carbinolamine-4a-dehydratase, cDcoH, DCoH, DCoH/PCD, DcoH2, DCoHalpha, Dehydratase, 4a-carbinolamine, Dimerization cofactor of hepatocyte nuclear factor 1-alpha, Dimerization cofactor of HNF1, Dimerization factor of HNF1/pterin-4alpha-carbinolamine dehydratase, GenBank AE000671-derived protein GI 2982796, More, P4aCD, PCD, PCD/DCoH, PCD/PhhB, PCDH, Phenylalanine hydroxylase-stimulating protein, Phenylalanine hydroxylase-stimulating protein/pterin-4alpha-carbinolamine dehydratase, PhhB, PHS, PHS/PCD, pterin 4a-carbinolamine dehydratase, Pterin carbinolamine dehydratase, Pterin-4 alpha-carbinolamine dehydratase, Pterin-4-alpha-carbinolamine dehydratase, Pterin-4a-carbinolamine dehydratase, Pterin-4a-carbinolamine dehydratase (Aquifex aeolicus gene phhB), Pterin-4a-carbinolamine dehydratase/dimerization cofactor of HNF1, Pterin-4alpha-carbinolamine dehydratase, Pterin-4alpha-carbinolamine dehydratase (PCD)/dimerization cofactor for the transcription factor HBF-1alpha, XDCoH
ECTree
4.2.1.96 4a-hydroxytetrahydrobiopterin dehydrataseAdvanced search results
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results (Natural Substrates Products
Natural Substrates Products on EC 4.2.1.96 - 4a-hydroxytetrahydrobiopterin dehydratase
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NATURAL SUBSTRATE 
NATURAL PRODUCT
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate)
(Substrate)
LITERATURE
(Substrate)
(Substrate)
COMMENTARY
(Product)
(Product)
LITERATURE
(Product)
(Product)
REVERSIBILITY
r=reversible
ir=irreversible
?=not specified
r=reversible
ir=irreversible
?=not specified
(6R)-6-(L-erythro-1,2-dihydroxypropyl)-5,6,7,8-tetrahydro-4a-hydroxypterin
(6R)-6-(L-erythro-1,2-dihydroxypropyl)-7,8-dihydro-6H-pterin + H2O
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additional information
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a defect in 4a-hydroxytetrahydrobiopterin dehydratase provoces the conversion of the 6-substituted pterins to their 7-substituted isomers
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additional information
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the mutant enzyme form C82R and the 18-amino acid-truncated mutant Glu87-termination occur naturally associated with hyperphenyalaninemia
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additional information
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the codevelopment of 4a-hydroxytetrahydropterin dehydratase with dihydropteridine reductase strongly supports a physiologically significant role for the dehydratase in tetrahydrobiopterin regeneration
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additional information
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the cytosolic enzyme is involved in the regeneration of tetrahydrobiopterin, the cofactor of aromatic amino acid monooxygenases
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additional information
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the enzyme is essentially identical to a cofactor that regulates dimerization of a nuclear homeodomain-containing protein involved in transcription
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additional information
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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
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additional information
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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
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additional information
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the enzyme may not play an important role in the regulation of the synthesis of those neurotransmitters which are derived from the hydroxylated aromatic amino acids
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additional information
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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
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additional information
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the cytosolic enzyme is involved in the regeneration of tetrahydrobiopterin, the cofactor of aromatic amino acid monooxygenases
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additional information
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the enzyme is essentially identical to a cofactor that regulates dimerization of a nuclear homeodomain-containing protein involved in transcription
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additional information
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enzyme plays a role in the regulation of expression of phenylalanine hydroxylase via transcription factor HNF1alpha
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?
