HTT and juvenile Huntington disease: It has been reported that defects in autophagy regulation, such as SQSTM1(p62) mutations [3], autophagy-related gene (Atg) 9 mislocation [4] or mutant huntingtin-mediated impairment of Beclin 1-mediated protein turnover [5,6], are associated with neurodegenerative disorders, including amyotrophic lateral sclerosis (ALS), Parkinson’s disease (PD) and Huntington’s disease (HD), respectively.