中文名稱    橋粒芯糖蛋白2抗體    

英文名稱    Desmoglein 2   

別    名    ARVC 10; ARVC10; ARVD 10; ARVD10; CDHF5; Desmoglein2; Desmoglein-2; DSG2; HDGC; HDGC included; DSG2_HUMAN; Desmoglein-2; Cadherin family member 5; HDGC.    

供 應 商   遠慕生物     

研究領域    心血管  免疫學  信號轉導  細胞粘附分子      

抗體來源    Rabbit    

克隆類型    Polyclonal    

交叉反應    Human, Mouse, Rat, Dog, Horse,     

產品應用    WB=1:100-500 ELISA=1:500-1000 IP=1:20-100 IHC-P=1:100-500 IHC-F=1:100-500 Flow-Cyt=1:100-500 ICC=1:100-500 IF=1:100-500橋粒芯糖蛋白2抗體 （石蠟切片需做抗原修復） 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.    

分 子 量    118kDa    

細胞定位    細胞膜     

性    狀    Lyophilized or Liquid    

濃    度    1mg/1ml    

免 疫 原    KLH conjugated synthetic peptide derived from human Desmoglein 2 (121-170aa)    

亞    型    IgG    

純化方法    affinity purified by Protein A    

儲 存 液    0.01M PBS, pH 7.4 with 10 mg/ml BSA and 0.1% Sodium azide    

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.    

橋粒芯糖蛋白2抗體產品介紹    background:

Desmosomes are cell-cell junctions between epithelial, myocardial, and certain other cell types. Desmoglein 2 is a calcium-binding transmembrane glycoprotein component of desmosomes in vertebrate epithelial cells. Currently, three desmoglein subfamily members have been identified and all are members of the cadherin cell adhesion molecule superfamily. These desmoglein gene family members are located in a cluster on chromosome 18. This second family member is expressed in colon, colon carcinoma, and other simple and stratified epithelial-derived cell lines.

Function:
Component of intercellular desmosome junctions. Involved in the interaction of plaque proteins and intermediate filaments mediating cell-cell adhesion.

Subcellular Location:
Cell membrane; Single-pass type I membrane protein. Cell junction, desmosome.

Tissue Specificity:
All of the tissues tested and carcinomas.

DISEASE:
Defects in DSG2 are the cause of familial arrhythmogenic right ventricular dysplasia type 10 (ARVD10) [MIM:610193]; also known as arrhythmogenic right ventricular cardiomyopathy 10 (ARVC10). 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. 
Genetic variations in DSG2 are the cause of susceptibility to cardiomyopathy dilated type 1BB (CMD1BB) [MIM:612877]. A disorder characterized by ventricular dilation and impaired systolic function, resulting in congestive heart failure and arrhythmia. Patients are at risk of premature death.