The treatment of mouse embryonic fibroblasts (MEFs) with IU1, a small-molecule inhibitor of USP14, improves the clearance of proteotoxic tau, TDP-43, ataxin-3 (ATXN3) and GFAP, which are disease-relevant proteins in AD, ALS, Machado–Joseph disease and glia overactivation, respectively (Lee et al. 2010). Here, ATXN3 is linked to amyotrophic lateral sclerosis.