Despite recent insight into UBQLN2’s role in diseases such as ALS-FTD (Hjerpe et al., 2016; Chang and Monteiro, 2015; Deng et al., 2011; Gorrie et al., 2014; Le et al., 2016), there is little understanding of how Ubqlns function in normal mammalian cell biology or physiology. The gene discussed is UBQLN2; the disease is amyotrophic lateral sclerosis.