Desmocollin 2 + 3,桥粒糖蛋白2/桥粒糖蛋白3抗体

产品编号HZ-2983R
英文名称Desmocollin 2 + 3
中文名称桥粒糖蛋白2/桥粒糖蛋白3抗体
别 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.
说 明 书0.1ml 0.2ml
研究领域心血管 细胞生物 信号转导 细胞粘附分子 细胞表面分子 细胞骨架 细胞外基质
抗体来源Rabbit
克隆类型Polyclonal
交叉反应Human, Mouse, Rat, Horse,
Desmocollin 2 + 3,桥粒糖蛋白2/桥粒糖蛋白3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84kDa
细胞定位细胞膜
性 状Lyophilized or Liquid
浓 度1mg/1ml
免 疫 原KLH conjugated synthetic peptide derived from human Desmocollin 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.
Desmocollin 2 + 3,桥粒糖蛋白2/桥粒糖蛋白3抗体PubMedPubMed
产品介绍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.

Desmocollin 2 + 3,桥粒糖蛋白2/桥粒糖蛋白3抗体Similarity:
Contains 5 cadherin domains.

Gene ID:
1824

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.