In neurodegenerative diseases, hyperphosphorylated tau fibrils (Jo et al., 2014), Aβ (Pickford et al., 2008), Htt-polyQ (Pickford et al., 2008), α-synuclein (Winslow et al., 2010), and TDP-43 (Buchan et al., 2013; Scotter et al., 2014) are aggrephagy substrates. Here, HTT is linked to neurodegenerative disease.