“…We focused on this apparent dysregulation of protein homeostasis in A-T cells because many of the more common neurodegenerative diseases in the human population are associated with aggregation of specific polypeptides in the brain, in some cases with clear causative links between the aggregation and neurotoxicity, and diverse model systems have confirmed these relationships (Bourdenx et al, 2017;Currais et al, 2017;Gidalevitz et al, 2006;Groh et al, 2017; Kikis et al, 2010;Ross and Poirier, 2004). In addition, several of the SCA ataxias, while associated with diverse genetic mutations, generate aggregation-prone mutant proteins that cause cerebellum-specific neurodegeneration similar to that observed in A-T (Buijsen et al, 2019;Klockgether et al, 2019).…”