It is already known that cytoplasmic accumulation of TDP-43 aggregates is one of the major characteristics of TDP-43 proteinopathy (Kim et al., 2014; Scotter et al., 2015; Shenouda et al., 2018), and this is a common pathological feature associated with many neurodegenerative diseases, such as Alzheimer’s disease (AD), frontotemporal lobar degeneration (FTLD), and amyotrophic lateral sclerosis (ALS) (Neumann et al., 2006; Mackenzie et al., 2011; Tremblay et al., 2011). Here, TARDBP is linked to frontotemporal dementia.