p62 is known to regulate the aggregation and degradation of proteins associated with neurodegenerative disease including alpha-synuclein as well as tau (which aggregates in Alzheimer’s disease), and huntingtin (which aggregates in Huntington’s disease) (Babu et al., 2005; Watanabe et al., 2012; Kurosawa et al., 2015; Tanji et al., 2015). Here, SQSTM1 is linked to juvenile Huntington disease.