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La 6-piruvoiltetrahidropterina sintasa (PT … La 6-piruvoiltetrahidropterina sintasa (PTS) (EC 4.2.3.12) es una enzima que cataliza una eliminación del grupo trifosfato y una reacción redox intramolecular en su sustrato. 7,8-dihidroneopterina-3'-trifosfato 6-piruvoil-5,6,7,8-tetrahidropterina + trifosfato Esta enzima participa en la síntesis de la tetrahidrobiopterina (BH4) a partir de GTP conjuntamente con las enzimas sepiapterina reductasa (EC 1.1.1.153) y GTP ciclohidrolasa I (EC 3.5.4.16). La tetrahidrobiopterina es un cofactor esencial de las hidrolasas de aminoácidos aromáticos: triptófano hidroxilasa (EC 1.14.16.4), fenilalanina hidroxilasa (EC 1.14.16.1) y tirosina hidroxilasa (EC 1.14.16.2). Se presenta como homohexámero formado por dos homotrímeros colocados cara a cara. El sitio activo está en la interfaz entre dos subunidades. El aceptor de protones cisteína está situado en una subunidad y el sistema de transmisión de carga en la otra subunidad. Como cofactor utiliza un ion zinc por subunidad. La enzima requiere para tener la máxima actividad la fosforilación del residuo Ser-19. Los defectos en PTS son causa de hiperfenilalaninemia por deficiencia en BH4 tipo A, llamada también deficiencia en PTS. Es un desorden autosomal recesivo caracterizado por una reducción de los neurotransmisores dopamina y serotonina. También se caracteriza por síntomas neurológicos severos.teriza por síntomas neurológicos severos.
, The enzyme 6-pyruvoyltetrahydropterin synt … The enzyme 6-pyruvoyltetrahydropterin synthase (EC 4.2.3.12, PTPS) catalyzes the following chemical reaction: 7,8-Dihydroneopterin 3′-triphosphate 6-pyruvoyltetrahydropterin + triphosphate This reaction is the second step (shown above) in the biosynthesis of tetrahydrobiopterin from GTP, which is used as a cofactor in the synthesis of aromatic amino acid monooxygenases and nitric oxide synthase PTPS converts 7,8-dihydroneopterin triphosphate to 6-pyruvoyltetrahydropterin (PTP) through the loss of the triphosphate group, a stereospecific reduction of the double bond between the top right nitrogen and carbon in the ring on the triphosphate on the right, the oxidation of the hydroxyl groups located on the first and second carbons of the side chain, and an internal base-catalyzed hydrogen transfer. ] 6-pyruvoyltetrahydropterin synthase (PTPS) can be found in the cytoplasm as well as the nucleus of cells according to immunohistochemical studies conducted. It has also been found that in higher species 6-pyruvoyltetrahydropterin synthase (PTPS) can undergo post-translational modification. This enzyme participates in tetrahydrobiopterin biosynthesis.pates in tetrahydrobiopterin biosynthesis.
, La 6-pyruvoyltétrahydroptérine synthase (P … La 6-pyruvoyltétrahydroptérine synthase (PTS) est une lyase qui catalyse la réaction :7,8-dihydronéoptérine 3'-triphosphate 6-pyruvoyl-5,6,7,8-tétrahydroptérine + triphosphate. Cette enzyme intervient dans la biosynthèse de novo de la tétrahydrobioptérine à partir du GTP en faisant intervenir la sépiaptérine réductase (EC 1.1.1.153) et la (EC 3.5.4.16). La réaction, catalysée en présence de cation de magnésium Mg2+, consiste en l'élimination d'un groupe triphosphate et en une réaction d'oxydoréduction intramoléculaire.éaction d'oxydoréduction intramoléculaire.
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| rdfs:comment |
La 6-piruvoiltetrahidropterina sintasa (PT … La 6-piruvoiltetrahidropterina sintasa (PTS) (EC 4.2.3.12) es una enzima que cataliza una eliminación del grupo trifosfato y una reacción redox intramolecular en su sustrato. 7,8-dihidroneopterina-3'-trifosfato 6-piruvoil-5,6,7,8-tetrahidropterina + trifosfato Los defectos en PTS son causa de hiperfenilalaninemia por deficiencia en BH4 tipo A, llamada también deficiencia en PTS. Es un desorden autosomal recesivo caracterizado por una reducción de los neurotransmisores dopamina y serotonina. También se caracteriza por síntomas neurológicos severos.teriza por síntomas neurológicos severos.
, La 6-pyruvoyltétrahydroptérine synthase (P … La 6-pyruvoyltétrahydroptérine synthase (PTS) est une lyase qui catalyse la réaction :7,8-dihydronéoptérine 3'-triphosphate 6-pyruvoyl-5,6,7,8-tétrahydroptérine + triphosphate. Cette enzyme intervient dans la biosynthèse de novo de la tétrahydrobioptérine à partir du GTP en faisant intervenir la sépiaptérine réductase (EC 1.1.1.153) et la (EC 3.5.4.16). La réaction, catalysée en présence de cation de magnésium Mg2+, consiste en l'élimination d'un groupe triphosphate et en une réaction d'oxydoréduction intramoléculaire.éaction d'oxydoréduction intramoléculaire.
, The enzyme 6-pyruvoyltetrahydropterin synt … The enzyme 6-pyruvoyltetrahydropterin synthase (EC 4.2.3.12, PTPS) catalyzes the following chemical reaction: 7,8-Dihydroneopterin 3′-triphosphate 6-pyruvoyltetrahydropterin + triphosphate This reaction is the second step (shown above) in the biosynthesis of tetrahydrobiopterin from GTP, which is used as a cofactor in the synthesis of aromatic amino acid monooxygenases and nitric oxide synthase PTPS converts 7,8-dihydroneopterin triphosphate to 6-pyruvoyltetrahydropterin (PTP) through the loss of the triphosphate group, a stereospecific reduction of the double bond between the top right nitrogen and carbon in the ring on the triphosphate on the right, the oxidation of the hydroxyl groups located on the first and second carbons of the side chain, and an internal base-catalyzed hydrogen trand an internal base-catalyzed hydrogen tra
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6-Pyruvoyltetrahydropterin synthase
, 6-piruvoiltetrahidropterina sintasa
, 6-Pyruvoyltétrahydroptérine synthase
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