RNF213, a cytosolic protein with a molecular weight of 591-kDa, is composed of two distinct functional domains, which exhibit six ATPase units and a multidomain E3 ubiquitin ligases module, and the pathologic mutations in MMD cluster in the E3 domain which not only result in a decline in the ubiquitin ligase activity of RNF213 but also boost the activation of NF-kappa B and apoptosis in an AAA + domain-dependent manner (8, 12, 13). Here, DNAH8 is linked to multiminicore myopathy.