Mice expressing the most aggressive mutation, FUSΔ14, recapitulated many aspects of human FUS proteinopathies, including insoluble FUS protein, basophilic and eosiniphilic neuronal cytoplasmic inclusions (NCI), and presence of other pathologic markers, including ubiquitin, p62/SQSTM1, α-internexin, and the polyadenylate-binding protein 1 (PABP-1). Here, FUS is linked to proteostasis deficiencies.