Detection Method:Sandwich
Test principle:This assay employs a two-site sandwich ELISA to quantitate PKP1 in samples. An antibody specific for PKP1 has been pre-coated onto a microplate. Standards and samples are pipetted into the wells and anyPKP1 present is bound by the immobilized antibody. After removing any unbound substances, a biotin-conjugated antibody specific for PKP1 is added to the wells. After washing, Streptavidin conjugated Horseradish Peroxidase (HRP) is added to the wells. Following a wash to remove any unbound avidin-enzyme reagent, a substrate solution is added to the wells and color develops in proportion to the amount of PKP1 bound in the initial step. The color development is stopped and the intensity of the color is measured.
Product Overview: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-null 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).
