It is well known that autophagy is a fundamental process to eliminate protein aggregations such as Aβ and tau in AD; α-synuclein (α-syn) in PD; huntingtin in Huntington’s disease (HD); or TAR DNA-binding protein 43 (TDP-43) in amyotrophic lateral sclerosis (ALS) [70]. This evidence concerns the gene TARDBP and juvenile Huntington disease.