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(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
4a(R)-hydroxy-6(S)-methyltetrahydropterin
quinoid 6(S)-methyldihydropterin
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4a(R)-hydroxy-6(S)-propyltetrahydropterin
quinoid 6(S)-propyldihydropterin
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4a(S)-hydroxy-6(R)-methyltetrahydropterin
quinoid 6(R)-methyldihydropterin
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4a-Hydroxy-6(R)-dihydroxypropyltetrahydropterin
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4a-Hydroxy-6(S)-methyltetrahydropterin
Quinoid 6(S)-methyldihydropterin
4a-Hydroxy-6(S)-propyltetrahydropterin
Quinoid 6(S)-propyldihydropterin
4a-hydroxy-tetrahydrobiopterin
7,8-dihydrobiopterin + H2O
4a-hydroxy-tetrahydropterin
7,8-dihydropterin + H2O
4a-hydroxytetrahydrobiopterin
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4alpha-hydroxy-6(S)-methyltetrahydropterin
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6(S)-methyl-4a-hydroxy-tetrahydropterin
6(S)-methyl-7,8-dihydropterin + H2O
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6(S)-methyl-7,8-dihydropterin-4a-carbinolamine
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6,6-dimethyl-4a-hydroxy-tetrahydropterin
6,6-dimethyl-7,8-dihydropterin + H2O
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pterin-4alpha-carbinolamine
quininoid dihydropterin + H2O
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additional information
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(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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(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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(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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4a-Hydroxy-6(S)-methyltetrahydropterin
Quinoid 6(S)-methyldihydropterin
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4a-Hydroxy-6(S)-methyltetrahydropterin
Quinoid 6(S)-methyldihydropterin
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4a-Hydroxy-6(S)-methyltetrahydropterin
Quinoid 6(S)-methyldihydropterin
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4a-Hydroxy-6(S)-propyltetrahydropterin
Quinoid 6(S)-propyldihydropterin
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4a-Hydroxy-6(S)-propyltetrahydropterin
Quinoid 6(S)-propyldihydropterin
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4a-hydroxy-tetrahydrobiopterin
7,8-dihydrobiopterin + H2O
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4a-hydroxy-tetrahydrobiopterin
7,8-dihydrobiopterin + H2O
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4a-hydroxy-tetrahydropterin
7,8-dihydropterin + H2O
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4a-hydroxy-tetrahydropterin
7,8-dihydropterin + H2O
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6(S)-methyl-7,8-dihydropterin-4a-carbinolamine
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6(S)-methyl-7,8-dihydropterin-4a-carbinolamine
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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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DCoHalpha is coactivator of HNF1alpha-depndent transcription
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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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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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4.2.1.96 4a-hydroxytetrahydrobiopterin dehydratase
results (
results (
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