TDP-43 and FUS, the nuclear proteins that bind RNA/DNA, are normally located in the neuronal nucleus, but relocate into the cytoplasm and colocalize with the ubiquitin-positive inclusion body in brains or spines of FTLD-U and ALS patients (Arai et al., 2006; Neumann et al., 2006). The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.