Transgenic or virus-directed expression of UBQLN2ALS mutants in rodents recapitulates protein UBQLN2 inclusions seen in ALS/FTD patients and elicits variable phenotypic abnormalities ranging to mild gait and memory defects to neuronal loss, paralysis, and early death (Gorrie et al., 2014; Wu et al., 2015; Huang et al., 2016; Le et al., 2016; Sharkey et al., 2020). The gene discussed is UBQLN2; the disease is frontotemporal dementia.