Recombinant Human PTS/PTPS Protein

重组人PTS/PTPS蛋白 | 重组人蛋白酪氨酸磷酸酶蛋白

6 pyruvoyl tetrahydrobiopterin synthase | 6 pyruvoyl tetrahydropterin synthase | 6 pyruvoyltetrahydropterin synthase | 6-pyruvoyl tetrahydrobiopterin synthase | EC 4.2.3.12 | FLJ97081 | OTTHUMP00000235385 | PTP synthase | PTPS | PTPS_HUMAN | PTS

rp188421-10μg

10μg

无载体

≥90%(SDS-PAGE)

PTS(6-pyruvoyltetrahydropterin synthase) belongs to the PTPS family. It catalyzes the elimination of inorganic triphosphate from dihydroneopterin triphosphate, which is the second and irreversible step in the biosynthesis of tetrahydrobiopterin from GTP. Tetrahydrobiopterin, also known as BH(4), is an essential cofactor and regulator of various enzyme activities, including enzymes involved in serotonin biosynthesis and NO synthase activity. Mutations in this gene result in hyperphenylalaninemia. PTS is involved in the biosynthesis of tetrahydrobiopterin, an essential cofactor of aromatic amino acid hydroxylases. PTS also catalyzes the transformation of 7,8-dihydroneopterin triphosphate into 6-pyruvoyl tetrahydropterin. Defects in PTS are the cause of BH4-deficient hyperphenylalaninemia type A (HPABH4A), also called 6-pyruvoyl-tetrahydropterin synthase deficiency (PTS deficiency) or hyperphenylalaninemia tetrahydrobiopterin-deficient due to PTS deficiency. HPABH4A is an autosomal recessive disorder characterized by depletion of the neurotransmitters dopamine and serotonin, and clinically by severe neurological symptoms unresponsive to the classic phenylalanine-low diet. Phosphorylation of Ser-19 is required for maximal enzyme activity.

重组表达

19.1 kDa

N-His

14.9 & 34.0 kDa, under reducing conditions; 15.0 & 33.2 kDa, under non-reducing conditions.

E. coli Accession #: Q03393 | E. coli

<1.0 EU/μg

人(Human)

2-145 aa

MHHHHHHSTEGGGRRCQAQVSRRISFSASHRLYSKFLSDEENLKLFGKCNNPNGHGHNYKVVVTVHGEIDPATGMVMNLADLKKYMEEAIMQPLDHKNLDMDVPYFADVVSTTENVAVYIWDNLQKVLPVGVLYKVKVYETDNNIVVYKGE

No

Yes