Whereas such hemichannels are predominantly closed under physiological conditions, they open under certain pathophysiological situations, such as in mdx mice (having a point mutation in the Dmd gene; a common mouse model of Duchenne muscular dystrophy), right ventricular arrhythmogenic cardiomyopathy in Plakophilin-2-(Pkp2)-deficient mice, and in ischemia/reperfusion (I/R) injury in mice [25,26,27]. Here, PKP2 is linked to Arrhythmogenic right ventricular dysplasia.