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    Polycystin 2 Rabbit pAb (bs-2158R)  
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    產(chǎn)品編號 bs-2158R
    英文名稱(chēng) Polycystin 2 Rabbit pAb
    中文名稱(chēng) 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.  
    研究領(lǐng)域 腫瘤  發(fā)育生物學(xué)  信號轉導  生長(cháng)因子和激素  新陳代謝  
    抗體來(lái)源 Rabbit
    克隆類(lèi)型 Polyclonal
    克 隆 號
    交叉反應 Human,Mouse (predicted: Rat,Cow,Chicken,Dog)
    產(chǎn)品應用 IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500
    not yet tested in other applications.
    optimal dilutions/concentrations should be determined by the end user.
    理論分子量 106 kDa
    檢測分子量
    細胞定位 細胞漿 細胞膜 
    性    狀 Liquid
    濃    度 1mg/ml
    免 疫 原 KLH conjugated synthetic peptide derived from human Polycystin 2: 651-750/968 
    亞    型 IgG
    純化方法 affinity purified by Protein A
    緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
    保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
    注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
    PubMed PubMed
    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.

    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



    多囊腎(polycystic kidney disease)為遺傳性疾病,是腎臟一種先天性異常。雙側腎臟皮髓質(zhì)均可累及,但在程度上可不同。在遺傳方式上表現為常染色體顯性和常染色體隱性遺傳兩種。
    囊內上皮細胞異常增殖是ADPKD的顯著(zhù)特特之一,處于一種成熟不完全或重發(fā)育狀態(tài),高度提示為細胞的發(fā)育成熟調控出現障礙,使細胞處于一種未成熟狀態(tài),從而顯示強增殖性。表現為細胞轉運密切相關(guān)的Na+-K+-ATP ase的亞單位組合,分布及活性表達的改變;細胞信號傳導異常以及離子轉運通道的變化。細胞外基質(zhì)異常增生是ADPKD第三種顯著(zhù)特征。目前許多研究已證明:這些異常均有與細胞生長(cháng)有關(guān)的活性因子的參與。但關(guān)鍵的異常環(huán)節和途徑尚未明了。因基因缺陷而致的細胞生長(cháng)改變和間質(zhì)形成異常,是本病的重要發(fā)病機制之一。
    產(chǎn)品圖片
    Tissue/cell: human kidney carcinoma; 4% Paraformaldehyde-fixed and paraffin-embedded; Antigen retrieval: citrate buffer ( 0.01M, pH 6.0 ), Boiling bathing for 15min; Block endogenous peroxidase by 3% Hydrogen peroxide for 30min; Blocking buffer (normal goat serum,C-0005) at 37℃ for 20 min; Incubation: Anti-Polycystin 2 Polyclonal Antibody, Unconjugated(bs-2158R) 1:200, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(C-0010) staining
    Paraformaldehyde-fixed, paraffin embedded (Mouse testis); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (Polycystin 2) Polyclonal Antibody, Unconjugated (bs-2158R) at 1:400 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
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