Aggresome formation is dependent on several molecules whose genomic mutation is linked to ALS and FTD, including VCP, p62 and dynein–dynactin (Johnston et al., 2002; Puls et al., 2003; Seibenhener et al., 2004), as well as the ubiquilin-2-related molecule, ubiquilin-1 (Heir et al., 2006). Here, UBQLN1 is linked to frontotemporal dementia.