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Rabbit Anti-Desmocollin 2 + 3/BF647 Conjugated antibody (bs-2983R-BF647)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價(jià)
產(chǎn)品編號(hào) bs-2983R-BF647
英文名稱 Rabbit Anti-Desmocollin 2 + 3/BF647 Conjugated antibody
中文名稱 BF647標(biāo)記的橋粒糖蛋白2/橋粒糖蛋白3抗體
別    名 ARVD11; Cadherin family member 2; CDHF2; Desmocollin 3; Desmocollin3; Desmocollin-2; Desmocollin-3; Desmocollin2; Desmosomal glycoprotein II and III; Desmosomal glycoprotein II; Desmosomal glycoprotein II/III; Desmosomal glycoprotein III; DG2; DGII/III; DKFZp686I11137; DSC 2; DSC2; DSC2_HUMAN; DSC3.  
規(guī)格價(jià)格 100ul/2980元 購(gòu)買        大包裝/詢價(jià)
說 明 書 100ul  
研究領(lǐng)域 心血管  細(xì)胞生物  信號(hào)轉(zhuǎn)導(dǎo)  細(xì)胞粘附分子  細(xì)胞表面分子  細(xì)胞骨架  細(xì)胞外基質(zhì)  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) (predicted: Human, Mouse, Rat, Horse, )
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 84kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Desmocollin 2
亞    型 IgG
純化方法 affinity purified by Protein A
儲(chǔ) 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:
The protein encoded by this gene is a calcium-dependent glycoprotein that is a member of the desmocollin subfamily of the cadherin superfamily. These desmosomal family members, along with the desmogleins, are found primarily in epithelial cells where they constitute the adhesive proteins of the desmosome cell-cell junction and are required for cell adhesion and desmosome formation. The desmosomal family members are arranged in two clusters on chromosome 18, occupying less than 650 kb combined. Mutations in this gene are associated with arrhythmogenic right ventricular dysplasia-11. Alternative splicing results in two transcript variants encoding distinct isoforms. [provided by RefSeq, Jul 2008]

Function:
Component of intercellular desmosome junctions. Involved in the interaction of plaque proteins and intermediate filaments mediating cell-cell adhesion. May contribute to epidermal cell positioning (stratification) by mediating differential adhesiveness between cells that express different isoforms.

Subunit:
Interacts with DSP, PKP2 and JUP.

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

Tissue Specificity:
In all epithelia tested and heart.

DISEASE:
Defects in DSC2 are the cause of familial arrhythmogenic right ventricular dysplasia type 11 (ARVD11) [MIM:610476]; also known as arrhythmogenic right ventricular cardiomyopathy 11 (ARVC11). 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:
Contains 5 cadherin domains.

Database links:

Entrez Gene: 1824 Human

Entrez Gene: 1825 Human

Omim: 125645 Human

Omim: 600271 Human

SwissProt: Q02487 Human

SwissProt: Q14574 Human



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

Involvement in disease:Defects in DSC2 are the cause of familial arrhythmogenic right ventricular dysplasia type 11 (ARVD11); also known as arrhythmogenic right ventricular cardiomyopathy 11 (ARVC11). 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.
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