PKP2, Polyclonal Antibody

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

Polyclonal

IgG

Rabbit

Western blot analysis of extracts of various cell lines, using PKP2 antibody. Immunofluorescence analysis of U20S cell using PKP2 antibody. Blue: DAPI for nuclear staining.

Affinity Purification

Liquid

1mg/ml (lot specific)

Store at -20 degree C (regular) and -80 degree C (long term). Avoid freeze / thaw cycles.

Small volumes of anti-PKP2 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.

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.

Western Blot (WB), Immunohistochemistry (IHC), Immunofluorescence (IF)

WB: 1:500 - 1:2000
IHC: 1:50- 1:200
IF: 1:50- 1:200

92,756 Da

plakophilin 2

plakophilin-2

Plakophilin-2

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]

plakophilin 2: May play a role in junctional plaques. Defects in PKP2 are the cause of familial arrhythmogenic right ventricular dysplasia type 9 (ARVD9); 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. Belongs to the beta-catenin family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Cell adhesion; Membrane protein, integral; Motility/polarity/chemotaxis

Chromosomal Location of Human Ortholog: 12p11.21

Cellular Component: adherens junction; cell junction; cell-cell adherens junction; desmosome; integral to membrane; intercellular junction; intermediate filament; nucleoplasm; plasma membrane

Molecular Function: alpha-catenin binding; intermediate filament binding; protein binding; protein complex scaffold; protein kinase C binding; sodium channel regulator activity

Biological Process: cell-cell adhesion; gap junction assembly; heart development; intermediate filament bundle assembly; lipid homeostasis; maintenance of organ identity; negative regulation of cell migration; negative regulation of cell proliferation; ventricular cardiac muscle morphogenesis

Disease: Arrhythmogenic Right Ventricular Dysplasia, Familial, 9

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