Few reports have also described FTD associated with mutations in the fused in sarcoma (FUS) (Broustal et al., 2010; Van Langenhove et al., 2010), dynactin (DCTN1) (Münch et al., 2005), sequestosome 1 (SQSTM1) (Fecto et al., 2011; Rubino et al., 2012; van der Zee et al., 2014), colony-stimulating factor 1 receptor (CFS1R) (Münch et al., 2005; Guerreiro et al., 2013b), triggering receptor expressed on myeloid cells (TREM2) (Borroni et al., 2013; Guerreiro, 2013), ubiquilin-2 (UBQLN2) (Gellera et al., 2013), and heterogeneous nuclear ribonucleoprotein A2B1 (hnRNPA2B1) genes (Kim et al., 2013). Here, SQSTM1 is linked to frontotemporal dementia.