Fig. 2.

Figure illustrating five pathways putatively causing pathological remodelling in ACM: 1) Deficits in EB-1 ID protein trafficking provoke disturbance of the ID morphology. 2) Pathological activation of the hippo pathway and suppression of Wnt/β-catenin signalling, caused by molecular remodelling of ID proteins, Plakoglobin nuclear translocation and GSK3β-ID anchoring, recapitulates the ARVC phenotype. 3) Remodelling of ID proteins by Ca²⁺-activated CaMKII. 4) Possible direct effect of increased intracellular Ca²⁺ concentrations on the ID structure. 5) Intracellular accumulation and degradation of misfolded PLN proteins in PLN-R14Del mutation carriers. GSK3β glycogen synthase kinase 3 beta