More notably, cytoplasmic mislocalization, aggregation, and fragmentation of RBPs, in particular TDP-43 (termed TDP-43 proteinopathies), have been regarded as a pathological hallmark of ALS or frontotemporal dementia (FTD, a disease sharing many genetic and pathological features with ALS; Neumann et al., 2006; Mackenzie et al., 2010). Here, TARDBP is linked to proteostasis deficiencies.