4a-hydroxytetrahydrobiopterin dehydratase

4a-hydroxytetrahydrobiopterin dehydratase
4a-hy·droxy·tet·ra·hy·dro·bi·op·ter·in de·hy·dra·tase (hi-drok″se-tet″rə-hi″dro-bi-opґtər-in de-hiґdrə-tās) [EC 4.2.1.96 ] an enzyme of the lyase class that catalyzes the dehydration of 4α-hydroxytetrahydrobiopterin, a step in the regeneration of the cofactor tetrahydrobiopterin. It is encoded by the PCDB gene (locus 10q22), mutation of which, an autosomal recessive trait, causes a transient form of hyperphenylalaninemia with associated primapterinuria. Called also pterin-4α-carbinolamine dehydratase (PCD). Written also 4α-hydroxytetrahydrobiopterin dehydratase.

Medical dictionary. 2011.

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  • 4a-hydroxytetrahydrobiopterin dehydratase — In enzymology, a 4a hydroxytetrahydrobiopterin dehydratase (EC number|4.2.1.96) is an enzyme that catalyzes the chemical reaction:(6R) 6 (L erythro 1,2 dihydroxypropyl) 5,6,7,8 tetrahydro 4a hydroxypterin ightleftharpoons (6R) 6 (L erythro 1,2… …   Wikipedia

  • pterin-4α-carbinolamine dehydratase — (PCD) pter·in 4α car·bin·ol·amine de·hy·dra·tase (PCD) (terґin kahr″bin olґə mēn de hiґdrə tās) 4a hydroxytetrahydrobiopterin dehydratase …   Medical dictionary

  • List of EC numbers (EC 4) — This list contains a list of EC numbers for the fourth group, EC 4, lyases, placed in numerical order as determined by the Nomenclature Committee of the International Union of Biochemistry and Molecular Biology.EC 4.1: Carbon Carbon LyasesEC… …   Wikipedia

  • Biopterin — Strukturformel Allgemeines Name Biopterin Andere Namen …   Deutsch Wikipedia

  • PCBD1 — Pterin 4 alpha carbinolamine dehydratase/dimerization cofactor of hepatocyte nuclear factor 1 alpha PDB rendering based on 1dch …   Wikipedia

  • Sapropterin — Strukturformel Allgemeines Freiname Sapropterin …   Deutsch Wikipedia

  • PCBD2 — Pterin 4 alpha carbinolamine dehydratase/dimerization cofactor of hepatocyte nuclear factor 1 alpha (TCF1) 2 Identifiers Symbols PCBD2; DCOH2; DCOHM; PHS2 External IDs …   Wikipedia

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  • primapterin — pri·map·ter·in (pre mapґtər in) [from Primael, the first patient in whom it was detected + pterin] an isomer of biopterin substituted at the 7 position; it accumulates in the urine in patients with a defect in 4a hydroxytetrahydrobiopterin… …   Medical dictionary

  • primapterinuria — pri·map·ter·in·uria (pre map″tər ĭ nuґre ə) urinary excretion of primapterin, as occurs in tetrahydrobiopterin deficiency that is caused by mutation of the gene encoding 4a hydroxytetrahydrobiopterin dehydratase …   Medical dictionary

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