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Molecular mechanisms of acantholysis in pemphigus. Desmoglein 1 and 3 are linked to the intermediate filament keratin network of the keratinocyte whereas E-cadherin links to the actin cytoskeleton. Pathogenic autoantibodies in PV and PF bind the ectodomain of Dsg3 or Dsg1 and trigger intracellular signaling that leads to acantholysis. Keratinocyte apoptosis also results from this immunopathological process.
