ALS and FTD-linked FUS mutations result in pathological aggregation of FUS, with varying levels of ubiquitination, in the cytoplasm and nucleus of neurons and glia throughout the affected regions of the cortex, brainstem and spinal cord (Kwiatkowski et al., 2009; Munoz et al., 2009; Vance et al., 2009; Suzuki et al., 2012). The gene discussed is FUS; the disease is frontotemporal dementia.