A large fraction of FXTAS hippocampal neurons contained inclusions, with ubiquitin, p62, or UBQLN2 aggregates present in 25, 20%, or 8%, of neurons (> 300) counted in each of 4 FXTAS cases, respectively (Fig. 1b, f, and j). The gene discussed is SQSTM1; the disease is fragile X-associated tremor/ataxia syndrome.