P2RX7 and neuromuscular disease caused by qualitative or quantitative defects of dysferlin: In the case of dysferlinopathy, as shown in Figure 5, dysferlin deficiency results in impaired membrane repair [6], and de novo expression of non-selective channels, such as connexin-based hemichannels, P2X7 receptors and transient receptor potential TRPV2 channels [56], which might contribute to an altered Ca2+ homeostasis, mitochondrial dysfunction [12] and ROS production [13].