FUS-proteinopathies are a group of genetically and clinically heterogeneous diseases that manifest in different manners depending on the region affected, such as motor neuron diseases (ALS-FUS) or various forms of dementia, including frontotemporal lobar degeneration with FUS pathology (FTLD-FUS), atypical FTLD with ubiquitin pathology (aFTLD-U), and other distinct forms of FTLD such as neuronal intermediate filament inclusion disease (NIFID) and basophilic inclusion body disease (BIBD)1. Here, FUS is linked to proteostasis deficiencies.