Dsg2, Cadherin family member 5, HDGC

For Research Use Only. Not for use in diagnostic procedures.

Monoclonal

Mouse IgG1

Mouse

1 ml (100 ug/ml) 0.2 um filtered antibody solution in PBS, containing 0.1% bovine serum albumin and 0.02% sodium azide.

Product should be stored at 4 degree C. Under recommended storage conditions, product is stable for at least one year. The exact expiry date is indicated on the label.

Small volumes of anti-DSG2 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Product Description: The monoclonal antibody 6D8 recognizes the extracellular domain of human desmoglein-2. Desmogleins and desmocollins are members of the cadherin family of transmembrane proteins that together make up the core of the desmosome, a structure that provides transmembrane strength to tissues undergoing mechanical stress. The desmosomal cadherins, desmogleins and desmocollins, mediate calcium-dependent cell-cell adhesion by forming homotypic and heterotypic interactions with one another. The multiprotein desmosomal complex also includes the cytoplasmic desmosomal plaque proteins plakoglobin, phakophilins, and desmoplakin, which bind to the intracellular domain of the desmogleins and function to anchor the keratin intermediate filament network to site of cellcell contacts.
In human, four desmogleins have been identified (Dsg14). Desmogleins are synthesized with a signal peptide that directs them to the endoplasmic reticulum and a proregion that is removed during protein processing. The mature protein includes four highly conserved extracellular domains (EC 14) and a fifth membrane proximal, more variable EC domain that is referred to as the “extracellular anchor domain. Desmoglein-2 is expressed on various cells including simple epithelia and myocardium, tumors and and many cell cultures.
Desmogleins play critical roles in cell adhesion and skin blistering diseases, as they are the target antigens of autoimmune antibodies and bacterial toxins. Desmosomal dysfunction has been implicated in a number of diseases, including striate palmoplantar keratoderma, skin fragility, and ectodermal dysplasia, and most recently arrhythmic right ventricular cardiomyopathy (ARVC).

F, Immunofluorescence (IF), Immunoprecipitation (IP), Western Blot

Application Notes: W: A reduced sample treatment (Laemmli buffer) and SDS-PAGE was used. The band size is ~165 kDa (Ref.5).
Application Use: For immune fluorescence and Western blotting, dilutions to be used depend on detection system applied. It is recommended that users test the reagent and determine their own optimal dilutions. The typical starting working dilution is 1:10.

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DSG2_HUMAN

Desmosomes are cell-cell junctions between epithelial, myocardial, and certain other cell types. This gene product 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. Mutations in this gene have been associated with arrhythmogenic right ventricular dysplasia, familial, 10. [provided by RefSeq, Jul 2008]

DSG2: Component of intercellular desmosome junctions. Involved in the interaction of plaque proteins and intermediate filaments mediating cell-cell adhesion. Defects in DSG2 are the cause of familial arrhythmogenic right ventricular dysplasia type 10 (ARVD10); 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). A disorder characterized by ventricular dilation and impaired systolic function, resulting in congestive heart failure and arrhythmia. Patients are at risk of premature death.

Protein type: Membrane protein, integral; Cell adhesion; Calcium-binding

Chromosomal Location of Human Ortholog: 18q12.1

Cellular Component: desmosome; cell surface; apical plasma membrane; integral to membrane; plasma membrane; intercellular junction; lateral plasma membrane

Molecular Function: calcium ion binding

Biological Process: maternal process involved in pregnancy; apoptosis; cell adhesion; response to progesterone stimulus; homophilic cell adhesion; cell structure disassembly during apoptosis

Disease: Cardiomyopathy, Dilated, 1bb; Arrhythmogenic Right Ventricular Dysplasia, Familial, 10

1. Wahl, J et al; Plakoglobin domains that define its association with the desmosomal cadherins and the classical cadherins: identification of unique and shared domains. J Cell Sci 1996, 109: 1143 2. Lewis, J et al; Cross-talk between adherens junctions and desmosomes depends on plakoglobin. J Cell Biol 1997, 136: 919
3. Wahl, J et al; Generation of monoclonal antibodies specific for desmoglein family members. Hybrid Hybridomics 2002, 21: 37
4. Ota, T et al; No involvement of IgG autoantibodies against extracellular domains of desmoglein 2 in paraneoplastic pemphigus or inflammatory bowel diseases. J Dermatol Sci 2003, 32: 137
5. Mahoney, M et al; Delineation of diversified desmoglein distribution in stratified squamous epithelia: implications in diseases. Exp Dermatol 2006, 15: 101
6. Nava, P et al; Desmoglein-2: a novel regulator of apoptosis in the intestinal epithelium. MBC 2007, 18: 4565
7. Keim, S et al; Generation and characterization of monoclonal antibodies against the proregion of human desmoglein-2. Hybridoma 2008, 27: 249.

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