Supporting this, postmortem studies revealed that the impaired interaction between SFPQ and FUS was a pathological change shared by the frontal, hippocampal, and spinal cord motor neurons among FTLD/ALS-TDP, PSP, and CBD patients; this finding was not observed in patients with AD and PiD and age-matched controls [80,137]; hence, impairment of SFPQ/FUS interaction has a disease specificity for ALS/FTLD-TDP and 4R-tauopathies. This evidence concerns the gene FUS and amyotrophic lateral sclerosis.