In addition to its involvement in primary TDP-43 proteinopathies, TDP-43 aggregation is recognized as a secondary pathology in neurodegenerative disorders such as AD [95], HD [96] or Lewy body disease [97] – as well as systemic diseases like inclusion body myopathy (IBM) [98, 99] – where TDP-43 pathology can occur alongside aggregation of other disease proteins [100]. This evidence concerns the gene TARDBP and inclusion body myositis.