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PTS Rabbit pAb (bs-19592R)  
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產(chǎn)品編號(hào) bs-19592R
英文名稱 PTS Rabbit pAb
中文名稱 6丙酮酰四氫蝶呤合成酶PTPS抗體
別    名 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.  
研究領(lǐng)域 腫瘤  細(xì)胞生物  神經(jīng)生物學(xué)  
抗體來(lái)源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) Human,Mouse,Rat (predicted: Rabbit,Pig,Sheep,Cow,Dog,Horse)
產(chǎn)品應(yīng)用 WB=1:500-2000,IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 16 kDa
檢測(cè)分子量
細(xì)胞定位 細(xì)胞漿 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human PTS: 91-145/145 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項(xiàng) This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產(chǎn)品介紹 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

Database links:

Entrez Gene: 5805 Human

GenBank: NP_000308.1 Human

Omim: 612719 Human

SwissProt: Q03393 Human

Unigene: 503860 Human



產(chǎn)品圖片
Sample: 293T(Human) Cell Lysate at 30 ug Primary: Anti-PTS?(bs-19592R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 16 kD Observed band size: 16 kD
Paraformaldehyde-fixed, paraffin embedded (Rat brain); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (PTS) Polyclonal Antibody, Unconjugated (bs-19592R) at 1:400 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
Paraformaldehyde-fixed, paraffin embedded (Mouse brain); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (PTS) Polyclonal Antibody, Unconjugated (bs-19592R) at 1:400 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
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