Dysregulation in any of the autophagy steps leads to the deposition of unfolded proteins or inclusion bodies in neurons and initiates the progression of neurodegenerative diseases such as Parkinson’s disease (α-synuclein), Alzheimer’s disease (Amyloid-β, neurofibrillary tangle), amyotrophic lateral sclerosis (SOD1, TDP 43) and Huntington’s disease (mutant Htt) [43,44] (Figure 3). This evidence concerns the gene HTT and juvenile Huntington disease.