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蛋白2抗体规格 英文名称  Anti-Polycystin 2

中文名称  蛋白2抗体规格 

别    名 polycystic kidney disease 2; TRPP2; APKD2, C030034P18RIK, MGC138466, MGC138468, PC2, PKD2 (includes EG:5311), PKD4, POLYCISTIN-2, POLYCYSTIN 2, RGD1559992, TRPP2; Polycystic kidney disease 2 protein homolog; PC2.

产品属性：

浓    度  1mg/1ml

规 格  0.2ml/200μg

抗体来源  Rabbit

克隆类型   polyclonal

交叉反应   Human, Mouse, Rat, Chicken, Dog, Cow

产品类型   一抗  

研究领域     发育生物学 信号转导 生长因子和激素

蛋白分子量  predicted molecular weight: 106kDa 

性    状   Lyophilized or Liquid

免 疫 原  KLH conjugated synthetic peptide derived from human Polycystin 2 C-terminus

亚    型  IgG

纯化方法   affinity purified by Protein A

储 存 液   0.01M PBS, pH 7.4 with 10 mg/ml BSA and 0.1% Sodium azide

蛋白2抗体规格 产品应用   WB=1:100-500 ELISA=1:500-1000 IP=1:20-100 IHC-P=1:100-500 IHC-F=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.  

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. 

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

产品介绍 This gene encodes a member of the polycystin protein family. The encoded protein is a multi-pass membrane protein that functions as a calcium permeable cation channel, and is involved in calcium transport and calcium signaling in renal epithelial cells. This protein interacts with polycystin 1, and they may be partners in a common signaling cascade involved in tubular morphogenesis. Mutations in this gene are associated with autosomal dominant polycystic kidney disease type 2. [provided by RefSeq, Mar 2011].

Function : Involved in fluid-flow mechanosensation by the primary cilium in renal epithelium. PKD1 and PKD2 may function through a common signaling pathway that is necessary for normal tubulogenesis. Acts as a regulator of cilium length, together with PKD1. The dynamic control of cilium length is essential in the regulation of mechanotransductive signaling. The cilium length response creates a negative feedback loop whereby fluid shear-mediated deflection of the primary cilium, which decreases intracellular cAMP, leads to cilium shortening and thus decreases flow-induced signaling. Functions as a calcium permeable cation channel.

Subunit : Forms homooligomers. Isoform 1 interacts with PKD1 while isoform 3 does not. PKD1 requires the presence of PKD2 for stable expression. Interacts with CD2AP. Interacts with HAX1. Interacts with NEK8. Part of a complex containing AKAP5, ADCY5, ADCY6 and PDE4C.

Subcellular Location : Membrane; Multi-pass membrane protein (Potential). Endoplasmic reticulum. Cell projection, cilium.

DISEASE : Polycystic kidney disease 2 (PKD2) [MIM:613095]: A disorder characterized by progressive formation and enlargement of cysts in both kidneys, typically leading to end-stage renal disease in adult life. Cysts also occurs in the liver and other organs. It represents approximately 15% of the cases of autosomal dominant polycystic kidney disease. PKD2 is clinically milder than PKD1 but it has a deleterious impact on overall life expectancy. Note=The disease is caused by mutations affecting the gene represented in this entry.

Similarity : Belongs to the polycystin family.

Contains 1 EF-hand domain.

Database links : NCBI Reference Sequence: NP_000288 UniProtKB/Swiss-Prot: Q13563

（polycystic kidney disease）为遗传性疾病，是*一种先天性异常。双侧*皮髓质均可累及，但在程度上可不同。在遗传方式上表现为常染色体显性和常染色体隐性遗传两种。

囊内上皮细胞异常增殖是ADPKD的显著特特之一，处于一种成熟不完全或重发育状态，高度提示为细胞的发育成熟调控出现障碍，使细胞处于一种未成熟状态，从而显示强增殖性。表现为细胞转运密切相关的Na+-K+-ATP ase的亚单位组合，分布及活性表达的改变；细胞信号传导异常以及离子转运通道的变化。细胞外基质异常增生是ADPKD第三种显著特征。目前许多研究已证明：这些异常均有与细胞生长有关的活性因子的参与。但关键的异常环节和途径尚未明了。因基因缺陷而致的细胞生长改变和间质形成异常，是本病的重要机制之一。

实验流程：

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