FITC标记的磷酸化6丙酮酰四氢蝶呤合成酶PTPS抗体
产品名称： FITC标记的磷酸化6丙酮酰四氢蝶呤合成酶PTPS抗体
英文名称： Anti-phospho-PTS (Ser19)/FITC
产品编号： HZ-19593R-FITC
产品价格： null
产品产地： 中国/上海
品牌商标： HZbscience
更新时间： 2023-08-17T10:24:20
使用范围： ICC=1:50-200 IF=1:50-200
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Rabbit Anti-phospho-PTS (Ser19)/FITC Conjugated antibody
FITC标记的磷酸化6丙酮酰四氢蝶呤合成酶PTPS抗体
| 英文名称 | Anti-phospho-PTS (Ser19)/FITC |
| 中文名称 | FITC标记的磷酸化6丙酮酰四氢蝶呤合成酶PTPS抗体 |
| 别 名 | PTS (phospho S19); p-PTS (phospho S19); 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. |
| 规格价格 | 100ul/2980元 购买 大包装/询价 |
| 说 明 书 | 100ul |
| 产品类型 | 磷酸化抗体 |
| 研究领域 | 肿瘤 细胞生物 神经生物学 信号转导 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, |
| 产品应用 | ICC=1:50-200 IF=1:50-200 not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 | 17kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated Synthesised acetylpeptide derived from human PTS around the acetylation site of Ser19 |
| 亚 型 | IgG |
| 纯化方法 | affinity purified by Protein A |
| 储 存 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
| 保存条件 | Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. |
| 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 for 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. 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 application |
该基因编码的酶催化从三磷酸二氢新喋呤中消除无机三磷酸，这是从GTP生物合成四氢生物喋呤的第二个不可逆步骤。四氢生物喋呤，又称BH（4），是多种酶活性的重要辅助因子和调节因子，包括参与5-羟色胺生物合成和NO合成酶活性的酶。该基因突变导致高苯丙氨酸血症。[ RefSeq，OCT 2008提供]