PKP2 blocking peptide

PKP2 Antibody (C-term) Blocking peptide

• Gene Names: PKP2; ARVD9
• Synonyms: PKP2; PKP2 Antibody (C-term) Blocking peptide; Plakophilin-2; PKP2 blocking peptide

• Form/Format: Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.
• Sequence Length: 881

• Cellular Location: Nucleus. Cell junction, desmosome. Note: Nuclear and associated with desmosomes
• Tissue Location: 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.
• Preparation and Storage: Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C.

Related Product Information for PKP2 blocking peptide

May play a role in junctional plaques.

NCBI and Uniprot Product Information

• NCBI GI #: 296452867
• NCBI GeneID: 5318
• UniProt Accession #: Q99959; Q4VC01; Q99960; A0AV37; B8QFA1; B8QGS6; B8QGS7; D3DUW9
• Molecular Weight: 92,756 Da
• NCBI Official Full Name: Plakophilin-2
• NCBI Official Synonym Full Names: plakophilin 2
• NCBI Official Symbol: PKP2
• NCBI Official Synonym Symbols: ARVD9
• NCBI Protein Information: plakophilin-2
• UniProt Protein Name: Plakophilin-2
• Protein Family: Plakophilin
• UniProt Gene Name: PKP2
• UniProt Entry Name: PKP2_HUMAN

NCBI Description

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]

Uniprot Description

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

Cellular Component: adherens junction; cell 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