6丙酮酰四氢蝶呤合成酶PTPS抗体
规格：1mg/1ml
英文名： PTS
别名： 6 pyruvoyl tetrahydrobiopterin synthase; 6 pyruvoyl tetrahydropterin synthase; 6 pyruvoyltetrahydropterin synthase; 6-pyruvoyl tetrahydrobiopterin synthase; EC 4.2.3.12; FLJ97081; OTTHUMP00000235385;
分子量： 16kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human PTS
交叉反应：Human,
细胞定位：
6丙酮酰四氢蝶呤合成酶PTPS抗体产品介绍：background: The enzyme encoded by this gene 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. [provided by RefSeq, Oct 2008] Function: Involved in the biosynthesis of tetrahydrobiopterin, an essential cofactor of aromatic amino acid hydroxylases. Catalyzes the transformation of 7,8-dihydroneopterin triphosphate into 6-pyruvoyl tetrahydropterin. Post-translational modifications: Phosphorylation of Ser-19 is required for6丙酮酰四氢蝶呤合成酶PTPS抗体 maximal enzyme activity. DISEASE: Defects in PTS are the cause of BH4-deficient hyperphenylalaninemia type A (HPABH4A) [MIM:261640]; 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. Similarity: Belongs to the PTPS family.6丙酮酰四氢蝶呤合成酶PTPS抗体 Gene ID: 5805 Database links: Entrez Gene: 5805 Human GenBank: NP_000308.1 Human Omim: 612719 Human SwissProt: Q03393 Human Unigene: 503860 Human Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
6丙酮酰四氢蝶呤合成酶PTPS抗体产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域：肿瘤 细胞生物 神经生物学
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid