Several studies have suggested that modestly elevated levels of TDP-43 expression are associated with TDP-43 proteinopathy in ALS and other diseases such as FTD and inclusion body myopathy, Paget’s disease of the bone and frontotemporal dementia (IBMPFD) [27–30]. Here, TARDBP is linked to inclusion body myopathy with Paget disease of bone and frontotemporal dementia.