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
PCD — pacer cardioverter defibrillator; papillary collecting duct; paraneoplastic cerebellar degeneration; paroxysmal cerebral dysrhythmia; percutaneous catheter drainage; pervasive developmental disorder; phosphate citrate dextrose; plasma cell… … Medical dictionary
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
