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Rabbit Anti-Plakophilin 2/BF350 Conjugated antibody (bs-11063R-BF350)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產(chǎn)品編號 bs-11063R-BF350
英文名稱1 Rabbit Anti-Plakophilin 2/BF350 Conjugated antibody
中文名稱 BF350標(biāo)記的橋粒斑菲素蛋白2抗體
別    名 ARVD 9; ARVD-9; ARVD9; PKP 2; PKP2; PKP-2; Plakophilin-2. Plakophilin2; PKP2_HUMAN.  
規(guī)格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領(lǐng)域 心血管  神經(jīng)生物學(xué)  細(xì)胞粘附分子  細(xì)胞骨架  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) (predicted: Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep, )
產(chǎn)品應(yīng)用 ICC=1:50-200 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 97kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Plakophilin 2
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol
保存條件 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.
產(chǎn)品介紹 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.

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



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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