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Rabbit Anti-PCBD1/Cy5.5 Conjugated antibody (bs-19905R-Cy5.5)
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说 明 书: 100ul  
100ul/2980.00元
大包装/询价
产品编号 bs-19905R-Cy5.5
英文名称 Rabbit Anti-PCBD1/Cy5.5 Conjugated antibody
中文名称 Cy5.5标记的蝶呤甲醇胺脱水酶1抗体
别    名 4 alpha hydroxy tetrahydropterin dehydratase; 4-alpha-hydroxy-tetrahydropterin dehydratase; 6 pyruvoyl tetrahydropterin synthase/dimerization cofactor of hepatocyte nuclear factor 1 alpha (TCF1); 6 pyruvoyl tetrahydropterin synthase/dimerization cofactor of hepatocyte nuclear factor 1 alpha; DCoH; Dimerization cofactor of hepatic nuclear factor 1 alpha; Dimerization cofactor of hepatocyte nuclear factor 1 alpha; Dimerization cofactor of hepatocyte nuclear factor 1-alpha; Dimerization cofactor of HNF1; Dimerizing cofactor for HNF1; PCBD 1; PCBD; PCBD1; PCD; Phenylalanine hydroxylase stimulating protein; Phenylalanine hydroxylase-stimulating protein; PHS; PHS_HUMAN; Pterin 4 alpha carbinolamine dehydratase; Pterin 4 alpha carbinolamine dehydratase/dimerization cofactor of hepatocyte nuclear factor 1 alpha (TCF1); Pterin 4 alpha carbinolamine dehydratase/dimerization cofactor of hepatocyte nuclear factor 1 alpha; Pterin carbinolamine dehydratase; Pterin-4-alpha-carbinolamine dehydratase.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
研究领域 肿瘤  细胞生物  信号转导  表观遗传学  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Mouse,  (predicted: Human, Rat, Cow, Rabbit, )
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 12kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human PCBD1
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:
This gene encodes a member of the pterin-4-alpha-carbinolamine dehydratase family. The encoded protein has been identified as a moonlighting protein based on its ability to perform mechanistically distinct functions. The encoded protein functions as both a dehydratase involved in tetrahydrobiopterin biosynthesis, and as a cofactor for HNF1A-dependent transcription. A deficiency of this enzyme leads to hyperphenylalaninemia. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Jan 2014]

Function:
Involved in tetrahydrobiopterin biosynthesis. Seems to both prevent the formation of 7-pterins and accelerate the formation of quinonoid-BH2. Coactivator for HNF1A-dependent transcription. Regulates the dimerization of homeodomain protein HNF1A and enhances its transcriptional activity.

Subcellular Location:
Cytoplasm. Nucleus. Cytoplasmic and/or nuclear.

DISEASE:
Defects in PCBD1 are the cause of BH4-deficient hyperphenylalaninemia type D (HPABH4D) [MIM:264070]; also known as hyperphenylalaninemia with primapterinuria. HPABH4D is characterized by the excretion of 7-substituted pterins in the urine of affected patients.

Similarity:
Belongs to the pterin-4-alpha-carbinolamine dehydratase family.

Database links:

Entrez Gene: 395729 Chicken

Entrez Gene: 5092 Human

Entrez Gene: 13180 Mouse

Entrez Gene: 29700 Rat

Omim: 126090 Human

SwissProt: P61457 Human

SwissProt: P61458 Mouse

SwissProt: P61459 Rat

Unigene: 3192 Human

Unigene: 39473 Mouse

Unigene: 81180 Rat



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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