Abstract: Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) are two closely related disorders with overlapping clinical, genetic, and neuropathological features, forming a continuous disease spectrum (Ling et al., 2013). The major pathological hallmark of ALS and FTD are the depletion from the nucleus of the RNA-binding proteins TAR DNA‐binding protein 43 (TDP-43) and FUsed in Sarcoma (FUS) and their abnormal accumulation in ubiquitin-positive cytoplasmic inclusions (Ling et al., 2013). TDP-43 and FUS, whose genetic mutations are associated with the familial forms of ALS-FTD, participate in the regulation of RNA maturation and DNA repair, key processes whose dysregulation is central in ALS-FTD pathogenesis, along with the impairment of protein homeostasis (proteostasis) (Ling et al., 2013).