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货号： bs-10975R 基本售价： 1380.0 元 规格： 100ul
- 规格：100ul
- 价格：1380.00元
- 规格：200ul
- 价格：2200.00元
产品信息
- 产品编号
- bs-10975R
- 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; PKD1_MOUSE.
- 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
- Mouse, Rat, Dog, Horse, Rabbit,
- 产品应用
- ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 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 mouse Polycystin 1:351-450/4293 <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:
O08852
Gene ID:
18763
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.