To elucidate this issue, it seems important to investigate whether changes of expression or intracellular localization of ATXN2 occur in the brains of FTLD-TDP cases, since intracellular mislocalization or depletion of other TDP-43-interacting RBPs (such as FUS, hnRNPA1, and hnRNPA2/B1) has been found in brains of patients with ALS and multisystem proteinopathies and are considered to be involved in neurodegeneration [22, 28, 41]. Here, TARDBP is linked to amyotrophic lateral sclerosis.