Quantitative sandwich ELISA for measuring Human Plakophilin-1 (PKP1) in samples from cell culture supernatants, serum, whole blood, plasma and other biological fluids.

• Additional information:Plakophilin-1 is a member of the arm-repeat (armadillo) and plakophilin gene families. Plakophilin proteins contain numerous armadillo repeats, localize to cell desmosomes and nuclei, and participate in linking cadherins to intermediate filaments in the cytoskeleton. This protein may be involved in molecular recruitment and stabilization during desmosome formation. Mutations in this gene have been associated with the ectodermal dysplasia/skin fragility syndrome.Compared with those in controls, desmosomes in PKP1- patients were reduced dramatically both in size (49%) and frequency (61%) in the lower suprabasal layers (P less than 0.01). In the lower suprabasal compartment of the heterozygous carrier, corresponding reductions were 37% and 20%, respectively (P less than 0.01).
• Storage and shipping:All of the components are shipped on ice packs/blue ice. Upon receiving the kit keep it at +4 degrees Celsius for up to 1 year (refer to the label on the box for the exact expiration date).
• Notes:This kit is intended for research use only. It should not be used for human or animal clinical diagnostics or treatment.