B6P, Band 6 protein, PKP1, Plakophilin-1 (ectodermal dysplasia/skin fragility syndrome)
Recognizes a protein of 75kDa, identified as Plakophilin-1. Its epitope maps between aa 1-27 human Plakophilin-1. 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 2 is important for desmosome assembly and is an essential morphogenic factor and architectural component of the heart.
