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蛋白2抗體規(guī)格 英文名稱(chēng)  Anti-Polycystin 2

中文名稱(chēng)  蛋白2抗體規(guī)格 

別    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.

產(chǎn)品屬性：

濃    度  1mg/1ml

規(guī) 格  0.2ml/200μg

抗體來(lái)源  Rabbit

克隆類(lèi)型   polyclonal

交叉反應(yīng)   Human, Mouse, Rat, Chicken, Dog, Cow

產(chǎn)品類(lèi)型   一抗  

研究領(lǐng)域     發(fā)育生物學(xué) 信號(hào)轉(zhuǎn)導(dǎo) 生長(zhǎng)因子和激素

蛋白分子量  predicted molecular weight: 106kDa 

性    狀   Lyophilized or Liquid

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

亞    型  IgG

純化方法   affinity purified by Protein A

儲(chǔ) 存 液   0.01M PBS, pH 7.4 with 10 mg/ml BSA and 0.1% Sodium azide

蛋白2抗體規(guī)格 產(chǎn)品應(yīng)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

（石蠟切片需做抗原修復(fù)） 

 not yet tested in other applications.

 optimal dilutions/concentrations should be determined by the end user.  

保存條件  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. 

產(chǎn)品介紹 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）為遺傳性疾病，是*一種先天性異常。雙側(cè)*皮髓質(zhì)均可累及，但在程度上可不同。在遺傳方式上表現(xiàn)為常染色體顯性和常染色體隱性遺傳兩種。

囊內(nèi)上皮細(xì)胞異常增殖是ADPKD的顯著特特之一，處于一種成熟不完全或重發(fā)育狀態(tài)，高度提示為細(xì)胞的發(fā)育成熟調(diào)控出現(xiàn)障礙，使細(xì)胞處于一種未成熟狀態(tài)，從而顯示強(qiáng)增殖性。表現(xiàn)為細(xì)胞轉(zhuǎn)運(yùn)密切相關(guān)的Na+-K+-ATP ase的亞單位組合，分布及活性表達(dá)的改變；細(xì)胞信號(hào)傳導(dǎo)異常以及離子轉(zhuǎn)運(yùn)通道的變化。細(xì)胞外基質(zhì)異常增生是ADPKD第三種顯著特征。目前許多研究已證明：這些異常均有與細(xì)胞生長(zhǎng)有關(guān)的活性因子的參與。但關(guān)鍵的異常環(huán)節(jié)和途徑尚未明了。因基因缺陷而致的細(xì)胞生長(zhǎng)改變和間質(zhì)形成異常，是本病的重要機(jī)制之一。

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