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 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 | |
1、抗体溶解方法 | |
2、抗体修复方式 | |
3、常用试剂的配制 | |
4、免疫组化操作步骤 | |
5、免疫组化问题解答 | |
6、Western Blotting 操作步骤 | |
7、Western Blotting 问题解答 | |
8、关于肽链的设计 | |
9、多肽的溶解与保存 | |
10、酶标抗体效价测定程序 | |