Anti-Plakophilin 1 Antibody, clone 10B2 clone 10B2, from mouse

Plakophilin-1 (UniProt: Q13835; also known as Band 6 protein, B6P) is encoded by the PKP1 gene (Gene ID: 5317) in human. Plakophilins are proteins that are involved in linking adherins to cytoskeletal intermediate filaments. They contain numerous armadillo (ARM) repeats and localize to cell desmosomes and nuclei. Plakophilin-1 may be involved in molecular recruitment and stabilization during desmosome formation. It is also shown to be important for tooth development and regulates ZO-1 distribution in the dental epithelium. Two isoforms of Plankophilin-1 have been reported that are produced by alternative splicing. Isoform 1 is expressed in stratified squamous, complex, glandular duct and bladder epithelia and isoform 2 is widely expressed. Plakophilin-1 is shown to be highly expressed in teeth and skin, and was upregulated during tooth development. It is reported to be involved in the regulation of Wnt signaling via its ARM repeat domains in a manner similar to beta-catenin and Wnt activation induces its nuclear translocation. Mutations n PKP1 gene are shown to cause ectodermal dysplasia-skin fragility syndrome that is characterized by cutaneous fragility and congenital ectodermal dysplasia affecting skin, hair and nails. (Ref.: Sprecher, E., et al (2004). J. Invest. Dermatol. 122(3); 647-51; Miyazaki, K et al. (2016). PLoS ONE 11(3): e0152206).