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Overview
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Background
Plakophilins 1, 2, 3 and 4 (PKP1-4) influence development and participate in linking cadherins to cytoskeletal intermediate filaments. Plakophilins 1-4 contain arm-repeat (armadillo) domains and localize to nuclei and cell desmosomes (cell-cell junctions found in suprabasal layers of stratifying epithelia that undergo mechanical stress). Plakophilin 1 mediates increases in desmosomal protein content, desmosome assembly and regulation of cell migration. Plakophilin 2 is important for desmosome assembly and is an essential morphogenic factor and architectural component of the heart. Plakophilin 4 is a component of desmosomal adhesion plaques that regulates junctional plaque organization and cadherin function.
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