ATXN3 and neurodegenerative disease: Considering the evidence linking ATXN3 containing expanded CAG repeats to ER stress (Rodrigues et al., 2011; Evers et al., 2014; Costa and Paulson, 2012; Matos et al., 2011; Reina et al., 2010), and our previous findings connecting ER stress to other neurodegenerative diseases (Vaccaro et al., 2012a, 2013; Julien et al., 2016), we investigated whether pharmacological interventions centred on ER stress could protect against mutant ATXN3-CAG89 toxicity.