Plakophilin 2,桥粒斑菲素蛋白2抗体

产品编号HZ-11063R
英文名称Plakophilin 2
中文名称桥粒斑菲素蛋白2抗体
别 名ARVD 9; ARVD-9; ARVD9; PKP 2; PKP2; PKP-2; Plakophilin-2. Plakophilin2; PKP2_HUMAN.
说 明 书0.1ml 0.2ml
研究领域心血管 神经生物学 细胞粘附分子 细胞骨架
抗体来源Rabbit
克隆类型Polyclonal
交叉反应 Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep,
Plakophilin 2,桥粒斑菲素蛋白2抗体产品应用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.
分 子 量97kDa
细胞定位细胞核 细胞膜
性 状Lyophilized or Liquid
浓 度1mg/1ml
免 疫 原KLH conjugated synthetic peptide derived from human Plakophilin 2
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.
Plakophilin 2,桥粒斑菲素蛋白2抗体PubMedPubMed
产品介绍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 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.

Plakophilin 2,桥粒斑菲素蛋白2抗体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 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

Plakophilin 2,桥粒斑菲素蛋白2抗体Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. 

产品图片

Tissue/cell: human colon carcinoma; 4% Paraformaldehyde-fixed and paraffin-embedded;  Antigen retrieval: citrate buffer ( 0.01M, pH 6.0 ), Boiling bathing for 15min; Block endogenous peroxidase by 3% Hydrogen peroxide for 30min; Blocking buffer (normal goat serum,C-0005) at 37℃ for 20 min;  Incubation: Anti-Plakophilin 2 Polyclonal Antibody, Unconjugated(HZ-11063R) 1:200, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(C-0010) staining Tissue/cell: human kidney tissue; 4% Paraformaldehyde-fixed and paraffin-embedded;  Antigen retrieval: citrate buffer ( 0.01M, pH 6.0 ), Boiling bathing for 15min; Block endogenous peroxidase by 3% Hydrogen peroxide for 30min; Blocking buffer (normal goat serum,C-0005) at 37℃ for 20 min;  Incubation: Anti-Plakophilin 2 Polyclonal Antibody, Unconjugated(HZ-11063R) 1:200, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(C-0010) staining