Depending on the regions affected, FUS-proteinopathies can manifest as motor neuron disease such as ALS-FUS, or as various forms of dementia including frontotemporal lobar degeneration with FUS pathology (FTLD-FUS),atypical FTLD with ubiquitin pathology (aFTLD-U), neuronal intermediate filament inclusion disease (NIFID), and Basophilic Inclusion Body Disease (BIBD) [4,10,11,12,13,14]. Here, FUS is linked to frontotemporal dementia.