FUS, identified as a predominantly nuclear protein, accumulates and aggregates in the cytoplasm in both FUS-ALS and FUS-FTLD [43, 55] and the apparent increase in cytoplasmic staining in hFUS (+/+) mice suggests that FUS overexpression in these animals may significantly increase cytoplasmic FUS levels. The gene discussed is FUS; the disease is amyotrophic lateral sclerosis.