The impact of POPDC protein mislocalisation on cAMP signalosomes could explain the cardiac arrhythmias which have been observed in many patients carrying BVES or POPDC2 variants [5, 10, 11, 15, 19, 37, 40] and may also be responsible for the muscular dystrophy phenotype associated with POPDC mutations, although the link to cAMP signaling has not yet been established in the case of skeletal muscle. The gene discussed is POPDC2; the disease is cardiac arrhythmia.