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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

Reaction

4a-hydroxytetrahydrobiopterin
=
6,7-dihydrobiopterin
 +
H2O

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 Lyases
         4.2 Carbon-oxygen lyases
             4.2.1 Hydro-lyases
                4.2.1.96 4a-hydroxytetrahydrobiopterin dehydratase

Disease

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Disease on EC 4.2.1.96 - 4a-hydroxytetrahydrobiopterin dehydratase

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DISEASE
TITLE OF PUBLICATION
LINK TO PUBMED
4a-hydroxytetrahydrobiopterin dehydratase deficiency
7-substituted pterins in humans with suspected pterin-4a-carbinolamine dehydratase deficiency. Mechanism of formation via non-enzymatic transformation from 6-substituted pterins.
International database of tetrahydrobiopterin deficiencies.
Suspected pterin-4a-carbinolamine dehydratase deficiency: hyperphenylalaninaemia due to inhibition of phenylalanine hydroxylase by tetrahydro-7-biopterin.
[Screening of tetrahydrobiopterin deficiency among hyperphenylalaninemic patients]
6,7-dihydropteridine reductase deficiency
International database of tetrahydrobiopterin deficiencies.
6-pyruvoyltetrahydropterin synthase deficiency
International database of tetrahydrobiopterin deficiencies.
Colonic Neoplasms
Dimerization co-factor of hepatocyte nuclear factor 1/pterin-4alpha-carbinolamine dehydratase is necessary for pigmentation in Xenopus and overexpressed in primary human melanoma lesions.
Overexpression of pterin-4a-carbinolamine dehydratase/dimerization cofactor of hepatocyte nuclear factor 1 in human colon cancer.
Colorectal Neoplasms
Overexpression of pterin-4a-carbinolamine dehydratase/dimerization cofactor of hepatocyte nuclear factor 1 in human colon cancer.
Dehydration
Catalytic characterization of 4a-hydroxytetrahydropterin dehydratase.
Distribution of 4a-hydroxytetrahydropterin dehydratase in rat tissues. Comparison with the aromatic amino acid hydroxylases.
Evidence for the formation of the 4a-carbinolamine during the tyrosine-dependent oxidation of tetrahydrobiopterin by rat liver phenylalanine hydroxylase.
gtp cyclohydrolase i deficiency
International database of tetrahydrobiopterin deficiencies.
Melanoma
Dimerization co-factor of hepatocyte nuclear factor 1/pterin-4alpha-carbinolamine dehydratase is necessary for pigmentation in Xenopus and overexpressed in primary human melanoma lesions.
Neoplasms
Dimerization co-factor of hepatocyte nuclear factor 1/pterin-4alpha-carbinolamine dehydratase is necessary for pigmentation in Xenopus and overexpressed in primary human melanoma lesions.
Overexpression of pterin-4a-carbinolamine dehydratase/dimerization cofactor of hepatocyte nuclear factor 1 in human colon cancer.
Phenylketonurias
Four Years of Diagnostic Challenges with Tetrahydrobiopterin Deficiencies in Iranian Patients.
Homomeric and heteromeric interactions between wild-type and mutant phenylalanine hydroxylase subunits: evaluation of two-hybrid approaches for functional analysis of mutations causing hyperphenylalaninemia.
International database of tetrahydrobiopterin deficiencies.
Suspected pterin-4a-carbinolamine dehydratase deficiency: hyperphenylalaninaemia due to inhibition of phenylalanine hydroxylase by tetrahydro-7-biopterin.
Tetrahydrobiopterin and inherited hyperphenylalaninemias.
Vitiligo
Crystallization and preliminary crystallographic studies of recombinant dimerization cofactor of transcription factor HNF1/pterin-4 alpha-carbinolamine dehydratase from liver.
Defective tetrahydrobiopterin and catecholamine biosynthesis in the depigmentation disorder vitiligo.
Dimerization co-factor of hepatocyte nuclear factor 1/pterin-4alpha-carbinolamine dehydratase is necessary for pigmentation in Xenopus and overexpressed in primary human melanoma lesions.
Three-dimensional structure of the bifunctional protein PCD/DCoH, a cytoplasmic enzyme interacting with transcription factor HNF1.