Furthermore, recent discoveries of many ALS-FTD genes, including SQSTM1 (Fecto et al., 2011), OPTN (Maruyama et al., 2010; Wong and Holzbaur, 2014), TBK1 (Cirulli et al., 2015; Freischmidt et al., 2015; Pottier et al., 2015), and VCP (Johnson et al., 2010), that are involved in autophagy, indicates that autophagy dysfunction plays a pivotal role in ALS-FTD pathogenesis. This evidence concerns the gene SQSTM1 and frontotemporal dementia.