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Polycystin 1 Rabbit pAb (bs-2157R)  
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產品編號 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.  
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.  
研究領域 細胞生物  免疫學  發育生物學  
抗體來源 Rabbit
克隆類型 Polyclonal
克 隆 號
交叉反應 Human (predicted: Mouse)
產品應用 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
細胞定位 細胞膜 
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: 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



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的顯著特特之一,處于一種成熟不完全或重發育狀態,高度提示為細胞的發育成熟調控出現障礙,使細胞處于一種未成熟狀態,從而顯示強增殖性。表現為細胞轉運密切相關的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:這些異常均有與細胞生長有關的活性因子的參與。但關鍵的異常環節和途徑尚未明了。因基因缺陷而致的細胞生長改變和間質形成異常,是本病的重要發病機制之一。
產品圖片
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. Protocol The cells were then incubated in 5%BSA to block non-specific protein-protein interactions for 30 min at at room temperature .Cells stained with Primary Antibody for 30 min at room temperature. The secondary antibody used for 40 min at room temperature. Acquisition of 20,000 events was performed.
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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