Human Plakophilin 2, PKP2 ELI

Plakophilin-2 is a protein that in humans is encoded by the PKP2 gene.

Plakophilin 2 is expressed in skin and cardiac muscle, where it functions to link cadherins to intermediate filaments in the cytoskeleton.

In cardiac muscle, plakophilin-2 is found in desmosome structures located within intercalated discs.

Mutations in PKP2 have been shown to be causal in arrhythmogenic right ventricular cardiomyopathy.