桥粒斑菲素蛋白2抗体
规格：1尘驳/1尘濒
英文名： Plakophilin 2
别名： ARVD 9; ARVD-9; ARVD9; PKP 2; PKP2; PKP-2; Plakophilin-2. Plakophilin2; PKP2_HUMAN.
分子量： 97kDa
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 human Plakophi
交叉反应：Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep,
细胞定位：细胞核 细胞膜
桥粒斑菲素蛋白2抗体产物介绍：background: Plakophilins 1, 2, 3 and 4 (PKP1-4) influence development and participate in linking cadherins to cytoskeletal intermediate filaments. Plakophilins 1-4 contain arm-repeat (armadillo) domains, and localize to nuclei and cell desmosomes (cell-cell junctions found in suprabasal layers of stratifying epithelia that undergo mechanical stress). Plakophilin-1 mediates increases in desmosomal protein content, desmosome assembly, and regulation of cell migration. Plakophilin-2 is important for desmosome assembly and is an essential morphogenic factor and architectural component of the heart. Plakophilin-3 plays a role in both desmosome-dependent adhesion and signaling pathways. Plakophilin-4 is a component of desmosomal adhesion plaques that regulates junctional plaque organization and cadherin桥粒斑菲素蛋白2抗体 function. Function: May play a role in junctional plaques. Subunit: Interacts with DSC2. Subcellular Location: Nucleus. Cell junction, desmosome.Note=Nuclear and associated with desmosomes. Tissue Specificity: Widely expressed. Found at desmosomal plaques in simple and stratified epithelia and in non-epithelial tissues such as myocardium and lymph node follicles. In most stratified epithelia found in the desmosomes of the basal cell layer and seems to be absent from suprabasal strata. Post-translational modifications: Phosphorylated upon DNA damage, probably by ATM or ATR. DISEASE: Defects in PKP2 are the cause of familial arrhythmogenic right ventricular dysplasia type 9 (ARVD9) [MIM:609040]; also known桥粒斑菲素蛋白2抗体 as arrhythmogenic right ventricular cardiomyopathy 9 (ARVC9). ARVD is an autosomal dominant disease characterized by partial degeneration of the myocardium of the right ventricle, electrical instability, and sudden death. It is clinically defined by electrocardiographic and angiographic criteria; pathologic findings, replacement of ventricular myocardium with fatty and fibrous elements, preferentially involve the right ventricular free wall. Similarity: Belongs to the beta-catenin family. Contains 8 ARM repeats. Database links: Entrez Gene: 537784 Cow Entrez Gene: 486613 Dog Entrez Gene: 101150898 Gorilla Entrez Gene: 101830795 Hamster Entrez Gene: 100070065 Horse Entrez Gene: 5318 Human Entrez Gene: 67451 Mouse Entrez Gene: 100347738 Rabbit Entrez Gene: 287925 Rat Omim: 602861 Human SwissProt: Q99959 Human Unigene: 164384 Human Unigene: 2252 Mouse Unigene: 27944 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
桥粒斑菲素蛋白2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：心血管 神经生物学 细胞粘附分子 细胞骨架
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid