In addition, mutations in ubiquilin-2 (Deng et al., 2011), sequestosome-1 (Fecto et al., 2011) or another degradation pathway adaptor protein, valosin-containing protein (VCP) (Watts et al., 2004), can cause ALS and FTD characterised by TDP-43 aggregation. Here, VCP is linked to frontotemporal dementia.