Pathological studies in ALS/FTD have found that sense and antisense RNA foci as well as p62-positive and TDP-43-negative inclusions (DPRs), are widely distributed throughout the CNS, supporting the involvement of gain-of-function mechanisms (DeJesus-Hernandez et al., 2011; Gendron et al., 2013; Zu et al., 2013). Here, SQSTM1 is linked to frontotemporal dementia.