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Rabbit Anti-Plakophilin 2  antibody (bs-11063R)  
~~~促銷代碼KT202411~~~
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產品編號 bs-11063R
英文名稱 Rabbit Anti-Plakophilin 2  antibody
中文名稱 橋粒斑菲素蛋白2抗體
別    名 ARVD 9; ARVD-9; ARVD9; PKP 2; PKP2; PKP-2; Plakophilin-2. Plakophilin2; PKP2_HUMAN.  
Specific References  (1)     |     bs-11063R has been referenced in 1 publications.
[IF=5.08] Akdis, Deniz, et al. "Myocardial Expression Profiles of Candidate Molecules in Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia compared to Dilated Cardiomyopathy and Healthy Controls." Heart Rhythm (2015).  IHC-P ;  Human.  
研究領域 心血管  神經(jīng)生物學  細胞粘附分子  細胞骨架  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Human (predicted: Mouse,Rat,Rabbit,Pig,Sheep,Cow,Dog,Horse)
產品應用 IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 97kDa
細胞定位 細胞核 細胞膜 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Plakophilin 2: 801-881/881 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產品介紹 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.

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.

SWISS:
Q99959

Gene ID:
5318

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



產品圖片
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(bs-11063R) 1:200, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(C-0010) staining
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