Polycystin 1抗体特异性结合抗原：抗体本身不能直接溶解或杀伤带有特异抗原的靶细胞，通常需要补体或吞噬细胞等共同发挥效应以**病原微生物或导致病理损伤。然而，抗体可通过与病毒或**的特异性结合，直接发挥中和病毒的作用。
产品编号xy- 2157R
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.
JournalPMIDIFApplicationThe Journal of Neuroscience (2015)262459766.7470IHC-FCarcinogenesis (2014)233850635.6350IP
说 明 书100ul 200ul
研究领域细胞生物 **学 发育生物学
抗体来源Rabbit
克隆类型Polyclonal
Polycystin 1抗体交叉反应 Human, Mouse,
产品应用ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 Flow-Cyt=1μg/Test 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/1ml
免 疫 原KLH conjugated synthetic peptide derived from human Polycystin 1:131-230/4303 2<Extracellular>3
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.
Polycystin 1抗体保存条件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.
PubMedPubMed
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 ***** 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
Polycystin 1抗体（antibody,
Ab）是由效应B细胞（效应**B细胞）分泌，机体用于抵御外来物质，如病毒，**等抗原，结构呈“Y”字型的球状蛋白质，仅仅存在于脊椎动物的血液和B**细胞膜表面。凡是能够跟抗体结合的物质，均被称作抗原，因此对于抗抗体（能够结合抗体的抗体）来说，抗体本身也是一种抗原物质。
Polycystin 1抗体普通抗体重链和轻链的结构
重链结构：普通的**球蛋白具有2条重链（H链），分子量约为50kD,有μ、δ、γ、ε和α五种重链亚型，对应的**球蛋白名称分别为IgM、IgG、IgA、IgD和IgE。
轻链结构： 普通**球蛋白具有2条轻链（L链），分子质量约25kDa，有κ链和λ链两种亚型，这两种轻链决定了Ig的亚型类别（IgG1，IgG2，IgG3，IgG4）。一个天然的Ig分子两条轻链总是相同的，但在同一个体内可存在分别带有κ或λ链的抗体分子。不同种属生物体内两型轻链的比例不同，正常人血清**球蛋白κ链：λ链约为2：1，而在小鼠的比例为20：1。
2.2抗体Fab段和Fc段
IgG经木瓜蛋白酶酶切后裂解为2个完全相同的Fab段和1个Fc段,每个Fab段都为单价,可与抗原结合但不会再发生凝集反应；经胃蛋白酶酶切后裂解为1个完整F(ab)2片段和碎片化的Fc片段,F(ab’)2片段为双价,可同时结合两个抗原表位。Fab段为抗原结合片段（fragment of antigen binding，Fab），相当于抗体分子的两个臂，由一个完整的轻链和重链的VH和CH1结构域组成。Fc段为可结晶段（fragment crystallizable，Fc）相当于Ig的CH2和CH3结构域，是Ig与效应分子或者细胞相互作用的部位。Fab段包含完整的可变区，以及恒定区的CH1区域。Fc段仅指Ig恒定区CH2和CH3的区域，相当于Y字结构下面那一部分。
合格 SLC47A1 溶质载体蛋白家族47成员A1抗体
合格 MUL1 E3泛素连接酶MUL1抗体
合格 合格 BTBD1 BTB/POZ结构域蛋白1（丙型肝炎病毒的NS5A反转录蛋白8）抗体
合格 ANKRD17 锚蛋白重复结构域蛋白17抗体
合格 ARHGAP32 Rho GTP酶激活蛋白32抗体
合格 SASS6 纺锤体组装异常蛋白6抗体
合格 NEK9 丝氨酸/苏氨酸蛋白激酶NEK9抗体
合格 合格 LRCH1 富含亮氨酸重复家庭蛋白1抗体
合格 LRCH3 富含亮氨酸重复家庭蛋白3抗体
合格 合格 MARCH9 膜相关环指蛋白9抗体
合格 PSMC2/Proteasome 19S S7 蛋白酶体PRS7抗体
合格 合格 PSMA7 蛋白酶体PSMα7抗体
合格 Proteasome 20S beta 6 蛋白酶体PSMβ6抗体
合格 Proteasome 26S S2 肿瘤坏死因子受体相关蛋白2抗体
合格 PSMD8 蛋白酶调解因子8抗体
合格 PSMD7 蛋白酶调解因子7抗体
合格 RNF61/MKRN1 环指蛋白61抗体
合格 IGJ **球蛋白j链抗体
合格 ANKRD42 锚蛋白重复结构域蛋白42抗体
合格 CARKD 碳水化合物激酶结构域蛋白质抗体
合格 CC85C 卷曲螺旋域蛋白质85C抗体
合格 TSPAN6 四分子交联体6抗体（四旋蛋白）
合格 TSPAN13 四分子交联体13抗体（四旋蛋白）
合格 TSPAN20 四分子交联体20/四旋蛋白抗体
合格 TXNDC9 胚胎干细胞相关蛋白TXNDC9抗体
合格 HSPB7 热休克蛋白β7抗体