Model for role of PKP2 in PKC-regulated DP assembly. (A) Wild type. PKP2 recruits PKC to DP cytoplasmic complexes, where phosphorylation of DP at Ser2849 modulates its interaction with IF and allows DP assembly into cell–cell junctions. (B) PKP2 deficiency. PKC is no longer recruited to DP complexes and is free to phosphorylate other substrates, leading to aberrant accumulation of DP along IF and impaired DP assembly.