The abnormal aggregation of TDP-43 is also observed in other neurodegenerative diseases, including Perry syndrome [27, 44] and inclusion body myopathy associated with Paget disease of bone and frontotemporal dementia (IBMPFD) [30, 33], which are referred to as secondary TDP-43 proteinopathies. This evidence concerns the gene TARDBP and proteostasis deficiencies.