Furthermore, aberrant cytoplasmic aggregates of FUS protein in degenerating neurons have been observed in the brains and spinal cords of patients with FUS-linked ALS (1, 2), frontotemporal lobar degeneration (FTLD) (4, 5), neuronal intermediate filament inclusion disease (6), and basophilic inclusion body disease (7), collectively referred to as FUS proteinopathies (8, 9). This evidence concerns the gene FUS and frontotemporal dementia.