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Rabbit Anti-phospho-PTS (Ser19)/BF594 Conjugated antibody (bs-19593R-BF594)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-19593R-BF594
英文名稱1 Rabbit Anti-phospho-PTS (Ser19)/BF594 Conjugated antibody
中文名稱 BF594標記的磷酸化6丙酮酰四氫蝶呤合成酶PTPS抗體
別    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.  
規(guī)格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
產品類型 磷酸化抗體 
研究領域 腫瘤  細胞生物  神經生物學  信號轉導  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human, )
產品應用 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    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated Synthesised acetylpeptide derived from human PTS around the acetylation site of Ser19
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件 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.
產品介紹 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 applications.
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