Alleviation of autophagy is usually accompanied by an accumulation of SQSTM1/p62 mostly in large perinuclear aggregates or inclusion bodies which are also positive for ubiquitin, as reported in numerous neurodegenerative diseases (such as Alzheimer’s disease, Parkinson’s disease, and Huntington disease) [23]–[28]. This evidence concerns the gene SQSTM1 and juvenile Huntington disease.