FUS and amyotrophic lateral sclerosis: 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).