Plakophilin 2 (Pkp2) is an armadillo-repeat protein of the cardiac desmosome. The armadillo-repeat proteins contain ten 42-amino acid armadillo-repeat motifs. They are localized to the outer dense plaque of desmosomes and are involved in the bridging of desmosomal cadherins with desmoplakin and the intermediate filament system. Plakophilins are nuclear proteins that regulate transcription. Pkp2 interacts with several cell adhesion proteins and has two alternatively spliced isoforms (2a and 2b). The gene is mapped to human chromosome 12p11.
Synonyms: Anti-Plakophilin-2
Storage: -20C
Application: Anti-PKP2 antibody produced in rabbit, a Prestige Antibody, is developed and validated by the Human Protein Atlas (HPA) project (www.proteinatlas.org). Each antibody is tested by immunohistochemistry against hundreds of normal and disease tissues. These images can be viewed on the Human Protein Atlas (HPA) site by clicking on the Image Gallery link. The antibodies are also tested using immunofluorescence and western blotting. To view these protocols and other useful information about Prestige Antibodies and the HPA, visit sigma.com/prestige.
Biochem Physiol Actions: The gene PKP2 (plakophilin 2) encodes a desmosomal protein that belongs to the plakophilin family of proteins that are involved in desmosome formation and function. Desmosomal proteins participate in cell signaling. In mouse, Pkp2 is expressed mainly in the cardiac muscle and plays an important role in the heart development. Any mutation in this gene causes defects of heart morphogenesis and junctional architecture with embryonic lethality at midgestation. Mutations in this gene are also associated with arrhythmogenic right ventricular cardiomyopathy.
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