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Rabbit Anti-Polycystin 2/APC Conjugated antibody (bs-2158R-APC)
~~~促銷,代碼KT202502A~~~
~~~促銷,代碼KT202502B~~~
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價(jià)
產(chǎn)品編號(hào) bs-2158R-APC
英文名稱 Rabbit Anti-Polycystin 2/APC Conjugated antibody
中文名稱 APC標(biāo)記的多囊腎蛋白2抗體
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.  
規(guī)格價(jià)格 100ul/2980元 購買        大包裝/詢價(jià)
說 明 書 100ul  
研究領(lǐng)域 腫瘤  發(fā)育生物學(xué)  信號(hào)轉(zhuǎn)導(dǎo)  生長因子和激素  新陳代謝  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) Mouse,  (predicted: Human, Rat, Chicken, Dog, Cow, )
產(chǎn)品應(yīng)用 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 106kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Polycystin 2
亞    型 IgG
純化方法 affinity purified by Protein A
儲(chǔ) 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
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.
產(chǎn)品介紹 background:
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.

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



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

多囊腎(polycystic kidney disease)為遺傳性疾病,是腎臟一種先天性異常。雙側(cè)腎臟皮髓質(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)胞生長改變和間質(zhì)形成異常,是本病的重要發(fā)病機(jī)制之一。
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