产品货号 :  mlR19593

英文名称 :  phospho-PTS (Ser19)

中文名称 :  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.  

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 :  ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 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.

分 子 量 :  17kDa

细胞定位 :  细胞浆

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:RI(p-S)FS

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.

PubMed :  PubMed

产品介绍 :  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.

SWISS:

Q03393

Gene ID:

5805

Important Note:

This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.