ITT3784
ITT3784
- Catalog: ITT3784
- Gene/Protein: PKP2
- Product Description: Immunotag™ Plakophilin 2 Polyclonal Antibody
385.0000
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Antibody Specification | |
Datasheet | |
Target Protein | PlakophIL-in 2 |
Clonality | Polyclonal |
Storage/Stability | -20°C/1 year |
Application | WB,ELISA |
Recommended Dilution | Western Blot: 1/500 - 1/2000. ELISA: 1/20000. Not yet tested in other applications. |
Concentration | 1 mg/ml |
Reactive Species | Human,Rat |
Host Species | Rabbit |
Immunogen | The antiserum was produced against synthesized peptide derived from human PKP2. AA range:632-681 |
Specificity | Plakophilin 2 Polyclonal Antibody detects endogenous levels of Plakophilin 2 protein. |
Purification | The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen |
Form | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide. |
Gene Name | PKP2 |
Accession No. | Q99959 |
Alternate Names | PKP2; Plakophilin-2 |
Description | plakophilin 2(PKP2) Homo sapiens This gene encodes a member of the arm-repeat (armadillo) and plakophilin gene families. Plakophilin proteins contain numerous armadillo repeats, localize to cell desmosomes and nuclei, and participate in linking cadherins to intermediate filaments in the cytoskeleton. This gene product may regulate the signaling activity of beta-catenin. Two alternately spliced transcripts encoding two protein isoforms have been identified. A processed pseudogene with high similarity to this locus has been mapped to chromosome 12p13. [provided by RefSeq, Jul 2008], |
Cell Pathway/ Category | Arrhythmogenic right ventricular cardiomyopathy (ARVC), |
Protein Expression | Brain,Placenta, |
Subcellular Localization | nucleus,nucleoplasm,intermediate filament,plasma membrane,cell-cell junction,adherens junction,cell-cell adherens junction,intercalated disc,integral component of membrane,cell junction,desmosome, |
Protein Function | disease:Defects in PKP2 are the cause of familial arrhythmogenic right ventricular dysplasia 9 (ARVD9) [MIM:609040]; also known as arrhythmogenic right ventricular cardiomyopathy 9 (ARVC9). 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.,function:May play a role in junctional plaques.,PTM:Phosphorylated upon DNA damage, probably by ATM or ATR.,similarity:Belongs to the beta-catenin family.,similarity:Contains 8 ARM repeats.,subcellular location:Nuclear and associated with desmosomes.,tissue specificity:Widely expressed. Found at desmosomal plaques in simple and stratified epithelia and in non-epithelial tissues such as myocardium and lymph node follicles. In most stratified epithelia found in the desmosomes of the basal cell layer and seems to be absent from suprabasal strata., |
Usage | For Research Use Only! Not for diagnostic or therapeutic procedures. |