The HSPB8 pro-degradative activity was demonstrated with several different neuropathogenic proteins, like polyQ proteins (ARpolyQ, huntingtin-polyQ, ataxin-3-polyQ), beta-amyloid, alpha-synuclein, ALS proteins mutant SOD1 and TDP-43 fragments (Chávez Zobel et al., 2003; Wilhelmus et al., 2006; Carra et al., 2008a,b; Crippa et al., 2010b, 2016; Bruinsma et al., 2011; Seidel et al., 2012; Rusmini et al., 2013), and on five different RAN translated DPRs from the C9Orf72 gene linked to ALS and FTD (Cristofani et al., 2017b). This evidence concerns the gene ATXN3 and amyotrophic lateral sclerosis.