桥粒糖蛋白2/桥粒糖蛋白3抗体
规格:1mg/1ml
英文名: Desmocollin 2 + 3
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 glycoprotei
分子量: 84kDa
储存液:0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型:Polyclonal
亚型:IgG
纯化方法:affinity purified by Protein A
**原:KLH conjugated synthetic peptide derived from human Desmocol
交叉反应:Human, Mouse, Rat, Horse,
细胞定位:细胞膜
桥粒糖蛋白2/桥粒糖蛋白3抗体产品介绍: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, 桥粒糖蛋白2/桥粒糖蛋白3抗体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. 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桥粒糖蛋白2/桥粒糖蛋白3抗体 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.
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.
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
储存条件: Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源: Rabbit
外观: Lyophilized or Liquid