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Polycystin 2 Rabbit pAb (bs-24450R)  
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产品编号 bs-24450R
英文名称 Polycystin 2 Rabbit pAb
中文名称 多囊肾蛋白2抗体
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.  
研究领域 肿瘤  发育生物学  信号转导  生长因子和激素  新陈代谢  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human (predicted: Mouse,Rat,Rabbit,Pig,Cow,Zebrafish,Horse)
产品应用 WB=1:500-2000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理论分子量 106 kDa
检测分子量
细胞定位 细胞浆 细胞膜 
性    状 Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Polycystin 2: 41-140/968 <Cytoplasmic>
亚    型 IgG
纯化方法 affinity purified by Protein A
缓 冲 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存条件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事项 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
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.

Tissue Specificity:
Strongly expressed in ovary, fetal and adult kidney, testis, and small intestine. Not detected in peripheral leukocytes.

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.

SWISS:
Q13563

Gene ID:
5311

Database links:

Entrez Gene: 5311 Human

Entrez Gene: 353503 Rat

Omim: 173910 Human

SwissProt: Q13563 Human

SwissProt: O35245 Mouse

Unigene: 181272 Human

Unigene: 483692 Mouse

Unigene: 6442 Mouse



多囊肾(polycystic kidney disease)为遗传性疾病,是肾脏一种先天性异常。双侧肾脏皮髓质均可累及,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,是本病的重要发病机制之一。
产品图片
Sample: Lane 1: Panc-1 (Human) Cell Lysate at 30 ug Lane 2: A549 (Human) Cell Lysate at 30 ug Lane 3: Huvec (Human) Cell Lysate at 30 ug Lane 4: Siha (Human) Cell Lysate at 30 ug Primary: Anti-Polycystin 2 (bs-24450R) at 1/1000 dilution Secondary: IRDye8
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