多囊肾蛋白1抗体
规格：1尘驳/1尘濒
英文名： Polycystin 1
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.
分子量： 460kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：笔辞濒测肠濒辞苍补濒
亚型：滨驳骋
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from mouse Polycyst
交叉反应：Mouse, Rat, Dog, Horse, Rabbit,
细胞定位：细胞膜
多囊肾蛋白1抗体产物介绍：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 多囊肾蛋白1抗体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 life. Cysts also occurs in the liver and other organs. Its prevalence is estimated at about 1/1000. 多囊肾蛋白1抗体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. 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.
多囊肾蛋白1抗体产物应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 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.
研究领域：细胞生物 **学 发育生物学
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid