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货号： bs-19905R-AP 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-19905R-AP
- 英文名称
- Anti-PCBD1/AP
- 中文名称
- 碱性磷酸酶（AP）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
- Human, Mouse, Rat, Cow, Rabbit,
- 产品应用
- WB=1:50-200 IHC-P=1:50-200 IHC-F=1:50-200 ICC=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: 395729Chicken
Entrez Gene: 5092Human
Entrez Gene: 13180Mouse
Entrez Gene: 29700Rat
Omim: 126090Human
SwissProt: P61457Human
SwissProt: P61458Mouse
SwissProt: P61459Rat
Unigene: 3192Human
Unigene: 39473Mouse
Unigene: 81180Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.