Interestingly, strong evidence for a pathological role of TDP-43 in skeletal muscle is provided by the observation that TDP-43 aggregates, reminiscent of the aggregates in neurons, accumulate in patients with sporadic inclusion body myositis (sIBM), IBM associated with Paget’s disease of bone and frontotemporal dementia (IBMPFB), oculopharyngeal muscular dystrophy (OPMD), distal myopathies with rimmed vacuoles (DMRV) and myofibrillar myopathies, including desminopathy and myotilinopathy (Weihl et al., 2008; Küsters et al., 2009; Olivé et al., 2009; Vogler et al., 2018). This evidence concerns the gene TARDBP and oculopharyngeal muscular dystrophy.