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Rabbit Anti-Polycystin 1/BF647 Conjugated antibody (bs-2157R-BF647)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-2157R-BF647
英文名稱 Rabbit Anti-Polycystin 1/BF647 Conjugated antibody
中文名稱 BF647標記的多囊腎蛋白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.   
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
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
交叉反應 Human,  (predicted: Mouse, )
產品應用 Flow-Cyt=1:50-200 ICC=1:50-200 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 460kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Polycystin 1
亞    型 IgG
純化方法 affinity purified by Protein A
儲 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.
產品介紹 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.

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。
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