Later studies manifested that activation of autophagy exerts beneficial effects in almost all neurodegenerative diseases, primarily through the removal of cytoplasmic aggregates, such as the α-synuclein in PD, amyloid-β (Aβ) [18] and Tau protein [19] in AD, and the TAR DNA binding protein 43 (TDP-43) aggregates in amyotrophic lateral sclerosis (ALS) [20], mutations in autosomal dominant lateral temporal epilepsy (ADLTE) and Machado-Joseph disease (MJD) [21]or ataxin-3 mutants accumulated in spinocerebellar ataxia type 3 (SCA3) [22, 23]. Here, ATXN3 is linked to amyotrophic lateral sclerosis.