| 產(chǎn)品編號 | bs-2157R |
| 英文名稱 | Polycystin 1 Rabbit pAb |
| 中文名稱 | 多囊腎蛋白1抗體 |
| 別 名 | Autosomal dominant polycystic kidney disease protein 1; PBP; PKD; PKD1; Polycystic Kidney Disease 1; Polycystin 1 Precursor; PKD1; Pc-1; TRPP1; PKD1_HUMAN. |
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Specific References (5) | bs-2157R has been referenced in 5 publications.
[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.
[IF=6.721] Winokurow N et al. A role for polycystin-1 and polycystin-2 in neural progenitor cell differentiation.Cell Mol Life Sci. 2019 Mar 20. WB&IHC ; Mouse.
[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.
[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.
[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.
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| 研究領(lǐng)域 | 細(xì)胞生物 免疫學(xué) 發(fā)育生物學(xué) |
| 抗體來源 | Rabbit |
| 克隆類型 | Polyclonal |
| 交叉反應(yīng) | Human (predicted: Mouse) |
| 產(chǎn)品應(yīng)用 | Flow-Cyt=1μg/Test
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 理論分子量 | 460 kDa |
| 檢測分子量 | |
| 細(xì)胞定位 | 細(xì)胞膜 |
| 性 狀 | Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human Polycystin 1: 131-230/4303 <Extracellular> |
| 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 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: 5310 Human Entrez Gene: 18763 Mouse Omim: 601313 Human SwissProt: P98161 Human SwissProt: O08852 Mouse Unigene: 75813 Human Unigene: 290442 Mouse Unigene: 30435 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(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)胞信號傳導(dǎo)異常以及離子轉(zhuǎn)運通道的變化。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。 |
| 產(chǎn)品圖片 |
Blank control: SH-Sy5Y.
Primary Antibody (green line): Rabbit Anti-Polycystin 1 antibody (bs-2157R)
Dilution: 2μg /10^6 cells;
Isotype Control Antibody (orange line): Rabbit IgG .
Secondary Antibody : Goat anti-rabbit IgG-AF647
Dilution: 1μg /test.
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 cond
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