Examples of specific disease-associated aggregating proteins include amyloid-β (Glenner and Wong, 1984) and τ-protein (Grundke-Iqbal et al., 1986) in AD, α-synuclein in PD (Spillantini et al., 1997) and multiple system atrophy (Tu et al., 1998; Wakabayashi et al., 1998), poly-Q expansions in huntingtin protein in Huntington’s disease (DiFiglia et al., 1997), and either Cu/Zn superoxide dismutase (Rosen et al., 1993), TAR-DNA-binding protein of 43 kDa (TDP-43; Neumann et al., 2006), or fused in sarcoma (FUS; Kwiatkowski et al., 2009; Vance et al., 2009) in ALS. The gene discussed is FUS; the disease is juvenile Huntington disease.