Molecular weight (DA)
97kD
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.
Formulation
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Dilution rate
WB 1:500-2000;IHC-p 1:50-300; ELISA 2000-20000
Purification process (Immunogen)
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Background
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],
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
Protein name
Plakophilin-2
Abbreviation
Plakophilin 2
Other name
PKP2; Plakophilin-2
Fields
>>Arrhythmogenic right ventricular cardiomyopathy
Human protein sequence Database
Q99959
Mouse protein sequence database
Rat protein sequence database
Cellular localization
Nucleus . Cell junction, desmosome . Nuclear and associated with desmosomes.
Tissue expression
Detected in heart right ventricle (at protein level). 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.
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