Polycystin 1多囊肾蛋白1抗体

Polycystin 1多囊肾蛋白1抗体

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  • ¥1380 - 2200
  • LMAI Bio
  • LM-2157R
  • 进口/国产
  • 2025年07月13日
  • ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 Flow-Cyt=1μg/Test ICC=1:100-500 IF=1:100-500 (石蜡切片需做抗原修复)
  • Rabbit
  • Human, Mouse,
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    • 详细信息
    • 询价记录
    • 技术资料
    • 供应商

      上海联迈生物工程有限公司

    • 库存

      大量

    • 目录编号

      LM-2157R

    • 克隆性

      多克隆

    • 抗原来源

      Rabbit

    • 保质期

      1年

    • 抗体英文名

      Polycystin 1

    • 抗体名

      多囊肾蛋白1抗体

    • 宿主

      Rabbit

    • 适应物种

      Human, Mouse,

    • 免疫原

      KLH conjugated synthetic peptide derived from human Polycystin 1:131-230/4303 <Extracellular>

    • 亚型

      IgG

    • 形态

      Lyophilized or Liquid

    • 应用范围

      ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 Flow-Cyt=1μg/Test ICC=1:100-500 IF=1:100-500 (石蜡切片需做抗原修复)

    • 浓度

      1mg/ml

    • 保存条件

      Store at -20 °C

    • 规格

      100ul  200ul

    Polycystin 1多囊肾蛋白1抗体
    英文名称 Polycystin 1
    中文名称 多囊肾蛋白1抗体
    别    名 Autosomal dominant polycystic kidney disease protein 1; PBP; PKD; PKD1; Polycystic Kidney Disease 1; Polycystin 1 Precursor; PKD1; Pc-1; TRPP1; PKD1_HUMAN.   
    Polycystin 1多囊肾蛋白1抗体 Specific References  (4)     |     bs-2157R has been referenced in 4 publications.
    [IF=2.79] Ren, Jian-gang, et al. "Down-regulation of polycystin in lymphatic malformations: Possible role in the proliferation of lymphatic endothelial cells." Human Pathology (2017).  IHC-P ;  Human.  
    PubMed:28552828
    [IF=5.63] Chiou, Yi-Shiou, et al. "Peracetylated (−)-epigallocatechin-3-gallate (AcEGCG) potently prevents skin carcinogenesis by suppressing the PKD1-dependent signaling pathway in CD34+ skin stem cells and skin tumors." Carcinogenesis 34.6 (2013): 1315-1322.  IP ;  Mouse.  
    PubMed:23385063
    [IF=4.21] Kito, Yusuke, Chiemi Saigo, and Tamotsu Takeuchi. "Novel Transgenic Mouse Model of Polycystic Kidney Disease." The American Journal of Pathology (2017).  WB ;  Mouse.  
    PubMed:28666097
    [IF=6.75] Ohata, Shinya, et al. "Mechanosensory Genes Pkd1 and Pkd2 Contribute to the Planar Polarization of Brain Ventricular Epithelium." The Journal of Neuroscience 35.31 (2015): 11153-11168.  IHC-F ;  Mouse.  
    PubMed:26245976
    规格价格 100ul/1380元 购买    200ul/2200元 购买    大包装/询价
    说 明 书 100ul  200ul
    研究领域 细胞生物  免疫学  发育生物学  
    抗体来源 Rabbit
    克隆类型 Polyclonal
    交叉反应 Human, Mouse, 
    产品应用 ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 Flow-Cyt=1μg/Test ICC=1:100-500 IF=1:100-500 (石蜡切片需做抗原修复) 
    not yet tested in other applications.
    optimal dilutions/concentrations should be determined by the end user.
    分 子 量 460kDa
    细胞定位 细胞膜 
    性    状 Lyophilized or Liquid
    浓    度 1mg/ml
    免 疫 原 KLH conjugated synthetic peptide derived from human Polycystin 1:131-230/4303 <Extracellular>
    亚    型 IgG
    纯化方法 affinity purified by Protein A
    储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
    保存条件 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.
    PubMed PubMed
    产品介绍 background:
    This gene encodes a member of the polycystin protein family. The encoded glycoprotein contains a large N-terminal extracellular region, multiple transmembrane domains and a cytoplasmic C-tail. It is an integral membrane protein that functions as a regulator of calcium permeable cation channels and intracellular calcium homoeostasis. It is also involved in cell-cell/matrix interactions and may modulate G-protein-coupled signal-transduction pathways. It plays a role in renal tubular development, and mutations in this gene cause autosomal dominant polycystic kidney disease type 1 (ADPKD1). ADPKD1 is characterized by the growth of fluid-filled cysts that replace normal renal tissue and result in end-stage renal failure. Splice variants encoding different isoforms have been noted for this gene. Also, six pseudogenes, closely linked in a known duplicated region on chromosome 16p, have been described. [provided by RefSeq].

    Function:
    Involved in renal tubulogenesis. Involved in fluid-flow mechanosensation by the primary cilium in renal epithelium. Acts as a regulator of cilium length, together with PKD2. 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. May be an ion-channel regulator. Involved in adhesive protein-protein and protein-carbohydrate interactions.

    Subunit:
    Interacts with PKD2. Interacts with PRKX; involved in differentiation and controlled morphogenesis of the kidney. Interacts with NPHP1 (via SH3 domain).

    Subcellular Location:
    Membrane; Multi-pass membrane protein. Cell projection, cilium. Note=PKD1 localization to the plasma and ciliary membranes requires PKD2, is independent of PKD2 channel activity, and involves stimulation of PKD1 autoproteolytic cleavage at the GPS domain. 

    Post-translational modifications:
    After synthesis, undergoes cleavage between Leu-3048 and Thr-3049 in the GPS domain. Cleavage at the GPS domain occurs through a cis-autoproteolytic mechanism involving an ester-intermediate via N-O acyl rearrangement. This process takes place in the early secretory pathway, depends on initial N-glycosylation, and requires the REJ domain. There is evidence that cleavage at GPS domain is incomplete. Uncleaved and cleaved products may have different functions in vivo.

    DISEASE:
    Defects in PKD1 are the cause of polycystic kidney disease autosomal dominant type 1 (ADPKD1) [MIM:173900]. ADPKD is 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. Its prevalence is estimated at about 1/1000.

    Similarity:
    Contains 1 C-type lectin domain. 
    Contains 1 GPS domain. 
    Contains 1 LDL-receptor class A domain. 
    Contains 2 LRR (leucine-rich) repeats. 
    Contains 1 LRRCT domain. 
    Contains 1 LRRNT domain. 
    Contains 17 PKD domains. 
    Contains 1 PLAT domain. 
    Contains 1 REJ domain. 
    Contains 1 WSC domain. 

    SWISS:
    P98161

    Gene ID:
    5310

    Database links:

    Entrez Gene: 606755 Dog

    Entrez Gene: 5310 Human

    Entrez Gene: 18763 Mouse

    Entrez Gene: 24650 Rat

    Omim: 601313 Human

    SwissProt: P98161 Human

    SwissProt: O08852 Mouse

    Unigene: 75813 Human

    Unigene: 290442 Mouse

    Unigene: 30435 Mouse



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

    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 Polycystin 1多囊肾蛋白1抗体
    Blank control(blue): Hela(fixed with 2% paraformaldehyde(10 min)). 
    Primary Antibody:Rabbit Anti-Polycystin 1 antibody(bs-2157R), Dilution: 1μg in 100 μL 1X PBS containing 0.5% BSA; 
    Isotype Control Antibody: Rabbit IgG(orange) ,used under the same conditions ); 
    Secondary Antibody: Goat anti-rabbit IgG-PE(white blue), Dilution: 1:200 in 1 X PBS containing 0.5% BSA.

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