GTP cyclohydrolase I

GTP cyclohydrolase I
GTP cy·clo·hy·dro·lase I (siґklo-hiґdro-lās) [EC 3.5.4.16] an enzyme of the hydrolase class that catalyzes the first step in the biosynthesis of tetrahydrobiopterin from guanosine triphosphate. Deficiency of the enzyme, an autosomal recessive trait caused by mutation of the GCH1 gene encoding the enzyme, is a form of tetrahydrobiopterin deficiency (q.v.) with hyperphenylalaninemia; an autosomal dominant disorder with sex-influenced reduced penetrance caused by mutation of same gene manifests as a dopa-responsive dystonia without hyperphenylalaninemia, with reduced rate of tetrahydrobiopterin synthesis.

Medical dictionary. 2011.

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  • GTP cyclohydrolase I — (GTP 7,8 8,9 dihydrolase, EC number|3.5.4.16) is an enzyme that is part of the folate and biopterin biosynthesis pathways. It is responsible for the hydrolysis of guanosine triphosphate (GTP) to form 7,8 dihydroneopterin 3 triphosphate. PBB… …   Wikipedia

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  • GTP cyclohydrolase I — Vorhandene Strukturdaten: 1fb1 …   Deutsch Wikipedia

  • GTP cyclohydrolase II — In enzymology, a GTP cyclohydrolase II (EC number|3.5.4.25) is an enzyme that catalyzes the chemical reaction:GTP + 3 H2O ightleftharpoons formate + 2,5 diamino 6 hydroxy 4 (5 phosphoribosylamino)pyrimidine + diphosphateThus, the two substrates… …   Wikipedia

  • GTP cyclohydrolase IIa — In enzymology, a GTP cyclohydrolase IIa (EC number|3.5.4.29) is an enzyme that catalyzes the chemical reaction:GTP + 3 H2O ightleftharpoons 2 amino 5 formylamino 6 (5 phosphoribosylamino)pyrimidin 4(3H) one + 2 phosphateThus, the two substrates… …   Wikipedia

  • GTP ciclohidrolasa — Las GTP ciclohidrolasas son unas enzimas que catalizan la reacción de hidrólisis del GTP. Se conocen hasta la fecha tres GTP ciclohidrolasas: I, II y IIa. Se diferencian entre ellas por los productos de la reacción de hidrólisis. Estas enzimas… …   Wikipedia Español

  • GCHFR — GTP cyclohydrolase I feedback regulator, also known as GCHFR, is a human gene.cite web | title = Entrez Gene: GCHFR GTP cyclohydrolase I feedback regulator| url = http://www.ncbi.nlm.nih.gov/sites/entrez?Db=gene Cmd=ShowDetailView… …   Wikipedia

  • Hydrolase — Cristal de lysozyme Les hydrolases constituent une classe d enzymes qui catalysent les réactions d hydrolyse de molécules suivant la réaction générale : R R + H2O ⇌ R OH + R H On y trouve par exemple les estérases, qui hydrolysent les …   Wikipédia en Français

  • List of EC numbers (EC 3) — This list contains a list of EC numbers for the third group, EC 3, hydrolases, placed in numerical order as determined by the Nomenclature Committee of the International Union of Biochemistry and Molecular Biology.EC 3.1: Acting on Ester BondsEC… …   Wikipedia

  • EC 3.5 — Le groupe EC3.5 rassemble des enzymes appartenant à la famille des hydrolases. Elles ont pour particularité de catalyser des réactions d hydrolyse dans des molécules possédant un carbone et un azote liés par liaison simple, double ou triple. À la …   Wikipédia en Français

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