Description General description 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). Specificity Clone 10B2 specifically detects Planophilin-1 and targets an epitope with in the first 27 amino acids from the N-terminal region. Immunogen Epitope: N-terminus MBP-conjugated recombinant fragment corresponding to the first 235 amino acids from the N-terminal region of human Plakophilin-1. Application Anti-Plakophilin 1, clone 10B2, Cat. No. MABT1505, is a mouse monoclonal antibody that detects Plantophilin 1 and has been tested for use in Immunofluorescence and Western Blotting. Research Category Cell Structure Western Blotting Analysis: A representative lot detected Plakophilin 1 in Western Blotting applications (Pigors, M., et. al. (2011). Hum Mol Genet. 20(9):1811-9). Immunofluorescence Analysis: A representative lot detected Plakophilin 1 in Immunofluorescence applications (Sprecher, E., et. al. (2004). J Invest Dermatol. 122(3):647-51;Pigors, M., et. al. (2011). Hum Mol Genet. 20(9):1811-9). Target description ~80 kDa observed; 80.50 kDa calculated. Uncharacterized bands may be observed in some lysate(s). Physical form Format: Purified Protein G purified Purified mouse monoclonal antibody IgG1 in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide. Storage and Stability Stable for 1 year at 2-8°C from date of receipt. Disclaimer Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals. Quality Evaluated by Western Blotting in PC3 cell lysate. Western Blotting Analysis: 1 µg/mL of this antibody detected Plakophilin 1 in 10 µg of PC3 cell lysate. Other Notes Concentration: Please refer to lot specific datasheet.